Hypertriglyceridemia (HTG) is the third most common cause for acute pancreatitis (AP), and the incidence of hypertriglyceridemia induced acute pancreatitis (HTG-AP) is rising worldwide as a result of changes in lifestyle and dietary habits. In China, HTG accounts for 10-20% the causes of AP, even becoming the second leading cause of AP. The presentation and diagnosis of HTG-AP is similar to that of AP from other causes, however, the complication rates and severe AP are significantly higher in patients with HTG-AP than in patients with other etiologies. Regarding the management for HTG-AP, the initial management is similar to that of AP from other causes and mainly includes fluid resuscitation, pain control and nutritional support. In addition to supportive care, it is necessary to take appropriate measures to decrease serum TG levels, in order to alleviate the progression of AP and prevent recurrence. This review aims to summarize existing clinical and basic research evidence of HTG-AP, and seeks to highlight the epidemiology, definition, pathogenesis, clinical course, diagnosis, management, prevention of recurrence and specific clinical scenarios, such as HTG-AP during pregnancy, HTG-AP with diabetic ketoacidosis.