Although small bowel involvement by malignant melanoma is most frequently metastatic, a primary origin at this site has been reported in rare cases. The biological and clinical features of these cases have not yet been summarized. Here, another case of small bowel melanoma is described, and a review of the existing literature is given. An 81-year-old man presented with anemia and gastrointestinal bleeding. A tumor of the ileum was detected by video capsule endoscopy and removed by segment resection. Histological examination revealed a malignant melanoma of the ileum. Another primary site could be excluded by clinical and radiographic examination. The patient felt well five months after surgery. Only 18 cases of primary small bowel melanoma (including the present one) have been reported to date. A literature review reveals that this rare neoplasm is associated with a slight predilection for the male gender, a predilection for the ileum and a poor prognosis.