Abstract: Hyperlipidemic pancreatitis (HLP) is one of the most important causes of acute pancreatitis. The pathogenesis is associated with the accumulation of free fatty acids which can activate the inflammatory response. Compared with other causes, HLP is more likely to have the clinical features characterized by more severe symptoms, more complications, and higher likelihood of persistent organ failure. The HLP patients always need lipid lowering therapy such as apheresis or insulin therapy to reduce serum triglyceride rapidly. Assessment of the presence of hyperlipidemia in patients of acute pancreatitis and prompt management are crucial for the prognosis and the prevention of recurrence. This article reviews the etiology, pathogenesis, clinical features, and treatment of hypertriglyceridemia-induced acute pancreatitis.