Propofol infusion syndrome is a rare but very dangerous complication of propofol administration. Certain risk factors for developing propofol infusion syndrome are described as follows: Adequate propofol dose and duration, carbohydrate deficiency, severe illness, and concomitant administration of catecholamines and glucocorticosteroids. The pathophysiology of this condition includes impaired mitochondrial beta-oxidation of fatty acids, disruption of the electron transport chain, and blockage of beta-adrenergic receptors and cardiac calcium channels. The disease often presents as unexplained high anion gap metabolic acidosis, rhabdomyolysis, hyperkalemia, acute kidney injury, elevated liver enzymes, and cardiac dysfunction. Management of overt propofol infusion syndrome requires immediate cessation of propofol infusion and supportive care including hemodialysis, hemodynamic support, and extracorporeal membrane oxygenation in refractory cases. However, given the high mortality associated with propofol infusion syndrome, it must be emphasized that the best treatment is prophylaxis. Clinicians should consider and recommend alternative sedation regimens to long-term propofol infusion. Propofol is a sedative-hypnotic drug commonly used before surgery, before endotracheal intubation or other procedures, and as an inducer for sedation in intensive care units. Its use was approved by the Food and Drug Administration (FDA) in November 1989. Administration of propofol has many important advantages, including a rapid onset of action within seconds after administration and a short duration of action of up to 15 minutes. Propofol has sedative, anxiolytic and antispasmodic properties. In addition, propofol may have beneficial anti-inflammatory and antioxidant effects, as well as neuroprotective properties, including lowering intracranial pressure. Although the mechanism of action is not clear, propofol stimulates β-aminobutyric acid receptors, blocks N-methyl-D-aspartate receptors, and slows calcium influx through slow calcium ion channels. Seems to decrease Nonetheless, it has been shown that propofol is not without risks. The first reported death associated with propofol infusion was in 1990 in a 3-year-old girl in Denmark. This patient developed high anion gap metabolic acidosis (HAGMA), hypotension, and multiple organ failure. In 1992, we reported the deaths of five children with clinical presentations similar to the Danish case during propofol infusion. The term propofol infusion syndrome (PRIS) first appeared in the pediatric literature and was proposed by Bray, who reviewed 18 pediatric cases. The clinical spectrum of PRIS consists of bradycardia, cardiovascular collapse, HAGMA, rhabdomyolysis, hepatomegaly, and lipemia. Then, in 1996, the first case of lactic acidosis in an adult associated with propofol administration was reported. The patient was a 30-year-old woman admitted with an exacerbation of bronchial asthma who developed lactic acidosis of unknown etiology. Propofol infusion was discontinued and lactic acidosis resolved with good results. Unfortunately, in 1998, the first premature mortality associated with propofol use was reported in his 17-year-old male with intractable status epilepticus. The purpose of this work is to summarize the current state of knowledge about PRIS, primarily for adults (18+). First, we will confirm the etiology of PRIS. Next, we discuss the epidemiology of PRIS and proposed risk factors. Third, the clinical manifestations and diagnosis of PRIS are reviewed. Finally, we conclude by describing the prevention and treatment of established PRIS. Before considering the pathogenesis of PRIS, it is important to gain a basic understanding of energy metabolism. Under physiological conditions, glucose is the major source of energy for the brain, cardiac system, and skeletal muscle.