Hypertriglyceridemia (HTG) is a rare cause of pancreatitis. However, the relationship between acute pancreatitis and severe HTG is well recognized. We report a case of necrotizing pancreatitis due to severe HTG (type IV) in a patient with poorly controlled diabetes. It was of particular interest that serum pancreatic enzymes were normal even though the imaging studies indicated the presence of necrotizing pancreatitis. Our case clearly demonstrates the various indices of HTG-induced necrotizing pancreatitis with a normal pancreatic enzyme level despite there being a serum triglyceride level < or=1,000 mg/dL. We present this case with a review of literature for hyperlipidemic pancreatitis in Korea.