Hypertriglyceridemia is less frequent cause of acute pancreatitis. Serum triglyceride level of >1000–2000 mg/dl is identifiable risk factor. It presents as an episode of acute pancreatitis or recurrent acute pancreatitis. The mainstay of treatment includes restriction of fatty meal and starting with fibric acid derivatives. We report a case of 24-year-old female presenting with the second episode of acute pancreatitis. Her genetic analysis revealed the following mutations: lipoprotein lipase, transducin (beta)-like 2, apolipoprotein A-V, angiopoietin-like 3, and tribbles homolog 1.