Familial hypertriglyceridemia (familial dyslipidemia type IV) is a disorder characterized by overproduction of very low-density lipoproteins (VLDL) by the liver. As a result, patients have excess amounts of triglycerides and VLDL, which can cause acute pancreatitis. This article discusses the evaluation and treatment of familial hypertriglyceridemia and highlights the role of interdisciplinary collaboration in the evaluation and treatment of this condition.