Fibrolipomatous hamartoma of a nerve represents an uncommon soft-tissue tumor which typically involves the median nerve distribution near the wrist. Macrodactyly may or may not be present. The application of MRI using routine T1- and T2-weighted spin echo imaging sequences reveals serpiginous structures with low signal intensity. These structures probably represent the neural elements and associated perineural fibrosis. Further experience may allow for an accurate preoperative diagnosis of this tumor.