Lipofibromatous hamartoma is a rare, slow-growing, benign, fibrofatty tumor-like condition. The upper extremity nerves are commonly involved. It is characterized by thickened axonal bundles with epineural and perineural fibrosis and fatty infiltration around the axonal bundles. Macrodactyly is a characteristic symptom in one-third of cases. Treatment options are differentiated according to the symptomatology. The diagnosis can be made by ultrasonographic and magnetic resonance imaging findings for those obviating diagnostic biopsy.