Cutaneous leiomyoma is a benign dermal tumor of smooth muscle. Leiomyoma can occur at any age but typically develops during adolescence or early adult life. Congenital leiomyoma is an extremely rare reported variant. A five-month-old boy presented to our clinic with a progressively enlarging mass on his occipital scalp. Microscopic examination of the solitary ulcerated nodule showed a non-encapsulated mass in the dermis composed of spindle-shaped cells. The diagnosis was confirmed by immunohistochemical studies, which were compatible with leiomyoma. Therefore, clinical suspicion of leiomyoma should be considered in the case of a solitary ulcerated nodule in an infant.