Duodenal adenocarcinoma, a rare malignant lesion, is associated with a poor 5-year survival. Few series have addressed differences between resectable tumors of the proximal and distal duodenum. We reviewed records of 17 consecutive patients with adenocarcinoma of the duodenum who underwent resection: 10 had adenocarcinoma of the proximal duodenum, and seven had tumors of the distal duodenum. Most patients underwent pancreatoduodenectomy. Five patients with adenocarcinoma of the distal duodenum underwent segmental resection. No perioperative deaths occurred. Six of 10 patients with proximal tumors died of metastatic disease. Of the seven patients with tumors of the distal duodenum, five are alive without evidence of disease, and two died of unrelated causes. The survival of patients with adenocarcinoma of the distal duodenum is surprisingly good, and segmental resection is the procedure of choice.