Abstract
Dermatofibrosarcoma protuberans is a low-grade soft tissue sarcoma not often related to the head and neck area. Distant metastases are not often recorded. Wide surgical excision is considered the most appropriate treatment modality. In cases of insufficient or positive surgical margins, the role of adjuvant radiation therapy or targeted therapy might be beneficial. Dermatofibrosarcoma protuberans transformation to a neoplasm with fibrosarcomatous elements is extremely rare. The presence of fibrosarcomatous elements is associated with more aggressive behavior of the tumor and increases significantly its metastatic potential. We present a rare case of head and neck dermatofibrosarcoma protuberans with multiple local recurrences 13 years after the initial diagnosis.