Background: Small bowel cancer (SBC) is rare, however there is recent trend toward increased incidence.Methods: Retrospective evaluation of the clinical presentation, diagnosis, management and clinical outcome of adult patients with primary SBC who were admitted at Sohag University Hospital during 2014-2019.Results: Twenty-one patients with SBC were enrolled (14 males and 7 females) with median age of 56 (range 32-83) years. Small bowel mass was identified preoperatively in 13 patients (62%). Thirteen patients (62%) presented with abdominal emergencies after failure of prompt diagnosis of non-specific abdominal pain of variable durations (median: 4, range: 2-11 weeks). Apart from duodenal tumors, histopathological diagnosis for jejunal and ileal tumors was not achieved preoperatively. Tumor types were gastrointestinal stromal tumors, GISTs (9, 43%), lymphoma (8, 38%) and adenocarcinoma (4, 19%). Patients with jejunal and ileal masses were managed by resection and primary anastomosis. Duodenal tumors required local resection in 2 and pancreatico-duodenectomy in 4 patients. Increased risk of aggressive behavior in GISTs, advanced stage and incomplete resection in lymphomas and adenocarcinomas were associated with higher recurrence rates and diminished survival. Follow-up ranged from 5 to 48 months, with survival rate of 76% (16 patients alive).Conclusions: The diagnosis of SBC is difficult and delayed. Appropriate surgical management, even during emergency, could achieve prolonged survival.