Background: Dermatofibrosarcoma protuberans of the scalp (DFSP) is a rare soft tissue neoplasm originating from the dermal layer of the skin, usually affecting the adults.
Case report: The current case report presents a 48-year old male with a huge lump on the right side of parietal region. A wide local excision of the tumor was performed and the excised specimen was sent for histopathological examination. Histopathology and Immunohistochemistry was suggestive of DFSP.
Conclusion: Dermatofibrosarcoma protuberans is a rare neoplasm affecting the head and neck region. This unusual entity is more likely to recur when a small margin of surgical excision is performed. Wide local excision is the gold standard treatment and radiotherapy is preferred in recurrent diseases.