The small intestine is a frequent site for metastases of cutaneous or ocular melanoma. When the latter are absent, the diagnosis of primary intestinal melanoma can be proposed. Primary malignant melanoma of the small intestine arises from melanoblastic cells of the neural crests, which migrate to the distal ileum through the omphalomesenteric canal. Incomplete regression of the later leads to persistence of Meckel's diverticulum. We report herein a case of malignant melanoma of the small intestine without evidence of a cutaneous and/or ocular origin. Based on its location in the distal ileum, we propose that this tumor be classified as a primary malignant melanoma of the small intestine.