Retinoic acid has been used successfully as a differentiating agent in acute promyelocytic leukemia and neuroblastoma. However, some adverse effects have been recognized, such as headaches, dry skin and retinoic acid syndrome, a life threatening acute cardiorespiratory disorder. Acute pancreatitis with hyperlipidemia has rarely been reported. We experienced a case of acute pancreatitis with hyperlipidemia in a neuroblastoma patient after retinoic acid therapy for 21 months. Although the patient was ordered nothing by mouth and total parenteral nutrition was administrated, she died of disseminated intravascular coagulopathy and pulmonary hemorrhage, possibly because of oral intake during her recovery period.