This is the first report of the development of bone metastases in a patient with an insulinoma. Following the development of bone metastases, further chemotherapy with etoposide (VP-16) and cis-platinum was not successful and the patient's condition was rapidly fatal. This case demonstrates the unusually aggressive course and poor response to currently available chemotherapeutic agents of a malignant insulinoma. The clinical and diagnostic features of insulinomas are reviewed.