Hypertriglyceridemic acute pancreatitis is an emerging issue in gastroenterology, frequently underdiagnosed in clinical practice. Despite the rarity of the disease, hypertriglyceridemia should be considered as a leading cause of acute pancreatitis, especially in defined subsets of patients. Primary and secondary forms of hypertriglyceridemia need to be considered and excluded during the diagnostic work-up of all patients with acute pancreatitis. An accurate diagnosis is crucial to establish an appropriate treatment and to reduce the risk of recurrences. The aim of the present article is to briefly review epidemiology, etiology, diagnosis and therapy of hypertriglyceridemic acute pancreatitis, based on a clinical and practical point of view.