The term Propofol Infusion Syndrome (PRIS) was first coined by Bray in 1998. This article was intended to define some of the common clinical features of cases in which children are adversely affected. Propofol infusion syndrome (PRIS) is a rare but fatal side effect of commonly used drugs. Our aim was to review and correlate experimental and clinical data on this syndrome. Propofol infusion syndrome (PRIS) is a rare but potentially fatal side effect of propofol. There is no universally accepted definition, but most often describe various combinations of: Unexplained metabolic acidosis, rhabdomyolysis, hyperkalemia, hepatomegaly, renal failure, hyperlipidemia, arrhythmia, Brugada electrocardiogram (ECG; ST-segment elevation and convex T wave), and rapidly progressing heart failure. The first cases were reported in children in the early 1990s, with adult cases shortly thereafter. PRIS did not receive much attention until Cremer et al. published his landmark paper in The Lancet on his seven cases of PRIS in the neurosurgical intensive care unit, where he was the first to show that the cumulative dose of propofol was a major risk factor for its development. Emphasized on Months later, the US Food and Drug Administration (FDA) issued a warning against the use of propofol for long-term sedation in the pediatric population. In 2006, the FDA updated label information to limit the maximum recommended dose of propofol for sedation to 4 mg/kg per hour. At the same time, European regulators suggested monitoring patients for signs of metabolic acidosis, hyperkalemia, rhabdomyolysis, or elevated creatine kinase levels and/or heart failure. If either occurred, they recommended a dose reduction or discontinuation of propofol. Despite increased public awareness and regulatory action, PRIS continues to occur and kill. A total of 394 PRIS cases were registered between December 2001 and March 2015 in the European Database of Suspected Side Effects of Medicines operated by the European Medicines Agency, of which 137 (35%) resulted in death. . As previously mentioned, the pathogenesis of PRIS involves interactions between increased lipolysis, impaired fatty acid oxidation, mitochondrial dysfunction, underlying disease, and concomitant drug use (such as catecholamines and glucocorticosteroids). Common organ systems affected by PRIS include cardiovascular, liver, skeletal muscle, renal, and metabolic systems. Cardiovascular manifestations of PRIS include enlarged QRS complexes, Brugada syndrome-like patterns (particularly type 1), ventricular tachyarrhythmias, cardiogenic shock, and asystole. Skeletal muscle manifestations include myopathy and overt rhabdomyolysis. Skeletal muscle injury can be complicated by hyperkalemia and acute kidney injury. Metabolic symptoms of PRIS also include HAGMA (due to increased lactic acid). But other causes of elevated lactate, such as other forms of shock (septic, cardiogenic, etc.), tissue ischemia (bowel, limb), and certain drugs (epinephrine, beta-2 agonists, etc.) There is a possibility. Increased lactate level. Metabolic acidosis can further exacerbate hyperkalemia due to increased transcellular shift. Liver manifestations include elevated liver enzymes, hepatomegaly, and steatosis. It should be noted that PRIS has no specific signs or symptoms (other than propofol administration) and that its symptoms strongly overlap with other conditions that lead to serious illness (various forms of shock, kidney disease from other causes, etc.). It is wise to emphasize Therefore, when treating patients with possible PRIS, clinicians should bear in mind wide variations.