Purpose: Dermatofibrosarcoma protuberans is a rare tumor, accounting for 0.1% of all malignant tumors. Although metastasis is very uncommon, local recurrence occur frequently. Dermatofibrosarcoma protuberans occurring in children is even more rare; this is the first case report of congenital dermatofibrosarcoma protuberans in Korea. Methods: The patient is a 14-month-old male infant with a lesion that was first thought to be a birthmark. The lesion grew larger, and a punch biopsy revealed dermatofibrosarcoma protuberans. A wide local excision was performed with a 2 cm peripheral resection margin beyond the gross tumor lesion. Deep fascia and a portion of muscle underneath the central part of the lesion were also taken. The surgical defect was covered by a split-thickness skin graft. Results: There has been no clinical sign of recurrence over one year after the surgery. Conclusion: A patient with congenital dermatofibrosarcoma protuberans detected at an early stage underwent a wide local excision of the tumor after accurate diagnosis was carried out by biopsy and immunohistochemical studies. There was no clinical evidence of tumor recurrence during over a 1-year follow-up.