A case of rhabdomyoma of the spermatic cord in an infant with cryptorchidism is reported. This extremely rare tumor has not been described in an extrascrotal position or at such a young age. Differential diagnosis includes malignant rhabdomyosarcoma. Although fetal-type rhabdomyoma of the head and neck generally is a benign tumor, the prognosis of paratesticular fetal-type rhabdomyoma is unknown. Long-term follow-up is recommended.