A case of a small-bowel schwannoma with diffuse familiar lipomatosis is described. This case underlines the rarity of the neoplasm and its probably chance association with subcutaneous lipomatosis. The intestinal neoplasm was diagnosed preoperatively by upper gastrointestinal endoscopy and a small-bowel enema; computed tomography scan confirmed the intestinal lesion. Attention is focused on the morphological features of intestinal schwannomas and their biological behaviour.