Malignant stromal tumours of the duodenum are rare. The efficacy of surgical resection for duodenal leiomyosarcoma was assessed in 5 patients treated over an 11-year period, probably the largest series treated by a single surgeon. There were 3 women and 2 men with an age range of 27–52 years. Tumours were large (8.5–21 cm diameter) and partly cystic (4 cases). They arose from the second (2), third (2) and fourth parts of the duodenum. Resection was a major undertaking and comprised either partial duodenectomy (n = 4) or Whipple resection. Two patients required a right hemicolectomy in addition. Two patients with positive resection margins had adjuvant radiotherapy. Operative time ranged from 4.0 to 6.25 h and blood loss from 1.8 to 4.5 litres. Two patients developed complications: a transient low-output biliary fistula and an infected haematoma requiring percutaneous drainage. The 2 patients with incomplete resection died of recurrent disease at 3 and 15 months. The 3 survivors are free of disease at 24, 60 and 66 months. The results support a policy of aggressive resection despite the technical difficulties posed by these large and vascular tumours.