Macrodactylia Fibrolipomatosis Presenting as a Small Bowel Obstruction

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. She 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.