Fetal rhabdomyoma is a rare benign neoplasm of skeletal muscle that must be distinguished histologically from various malignant lesions, including rhabdomyosarcoma. They have recently been subdivided into two histological variants myxoid and cellular fetal rhabdomyomas. This article describes a case of an 8 month infant with a fetal rhabdomyoma in the upper lip. Illustrate the histological features and discusses the means of distinguishing these lesions from various other conditions with which they may be confused.