A congenital fetal rhabdomyoma was removed from the neck of a male infant on the second day of life. The lesion recurred 10 years later with histological features suggestive of increased differentiation and no evidence of malignant transformation. An unrelated patient of 3 years suffered from a recurrent fetal rhabdomyoma 4 months after the initial resection. The possibility of early or late recurrence must be considered when a diagnosis of fetal-type rhabdomyoma is made.