Primary malignant schwannoma of the small bowel is an extremely rare disease. Only 24 malignant schwannomas of the small bowel have been reported in the medical English literature. We report a primary malignant schwannoma of the distal ileum in a 53-year-old woman, who was admitted to the hospital because of abdominal pain. CT scan revealed a semisolid mass in the small intestine of the serosa. The tumour measuring 14 x 13 x 8 cm and 100 cm of the distal ileum were resected. We observed a recurrence after seventeen months and the recurrent tumour measured 6 x 2.5 x 1 cm was excised.