Leiomyosarcomas of the duodenum are rare tumors, usually presented with gastrointestinal bleeding and/or symptoms of duodenal obstruction, rarely with obstructive jaundice. If successfully removed, these tumors have better prognosis than carcinomas of duodenum or head of the pancreas. A 62 year old man in whom leiomyosarcoma of the duodenum was successfully removed by cephalic duodenopancreatectomy (Whipple's procedure) is presented.