Between 1959 and 1987 we operated on 18 patients for malignant oddian tumor. Eleven had a Whipple resection, 3 a bilio-enteric anastomosis, 4 a local excision with or without bilio-enteric anastomosis. The overall operative mortality was 11% and the median survival was 13.8 months. Three patients are living and without evidence of disease 12, 29 and 30 months, respectively, after a Whipple resection. Because of their anatomy and favourable behaviour, malignant oddian tumors must be separated from the other periampullary tumors. Echography and endoscopic retrograde cholangiopancreatography with deep biopsies are the most efficient diagnostic modalities. With the aim of cure, the treatment is always surgical and relies mainly on duodenopancreatectomy. Those patients with unresectable tumors or unfit for a major procedure should benefit from internal or external biliary drainage. By coexisting duodenal obstruction, a surgical double derivation should be done.