A patient presented with progressive abdominal distention, discomfort, nausea, vomiting, and constipation. The patient also had congenital macrodactyly of the third, fourth, and fifth digits of the left hand. He [corrected] was diagnosed with macrodactylia fibrolipomatosis with multiple small bowel lipomas and complete small bowel obstruction. Macrodactylia fibrolipomatosis is a rare condition characterized by benign fibrofatty infiltration involving the soft tissues of the distal arm or leg with associated dactylomegaly of the associated digits. This patient had a rare presentation that included extensive small bowel lipomatosis.