Desmoid tumor originating from the small intestine is extremely rare. We report a 50-year-old man who presented with the sudden onset of severe abdominal pain. Computerized tomography (CT) demonstrated a huge homogeneous tumor in the lower abdomen that appeared to be in continuity with the distal ileum. The mass adherent to the ileum was resected and proved to be a desmoid tumor. The patient has remained recurrence free on follow-up.