The small bowel is the most common site of gastrointestinal metastasis from cutaneous melanoma. Malignant melanoma has a poor prognosis, especially if distant metastases appear. Although rare primary melanoma of the small bowel has been described, more frequently these lesions originate from unknown cutaneous melanoma. Here we report the case of a 58-year-old man with a diagnosis of melanoma of the ileum without evidence of primary cutaneous disease. After 15 years, during the clinical and radiological follow-up, a cutaneous melanoma in the left parietal side of the scalp, probably corresponding to the primary lesion with abdominal node metastasis, was diagnosed. After 6 months of chemotherapy with fotemustine, the patient showed a complete response. At present, he is still alive 18 years after the diagnosis of intestinal metastasis.