Abstract
Dermatofibrosarcoma protuberans is a very rare tumour that accounts for less than 0.1% of all malignant neoplasms. It is a locally aggressive tumour with high recurrence rate. Head and neck involvement is very uncommon and hence diverse treatment protocols have been recommended for its management in the currently available literature. We present a case of dermatofibrosarcoma protuberans over the lower part of right cheek in a middle aged male, which was successfully treated with wide local excision and reconstruction along with post-op radiotherapy. The patient was followed-up for 18 months with no recurrence. Prompt and accurate diagnosis along with multidisciplinary treatment is crucial for optimal management of this rare tumor. The optimal treatment option is surgical resection with wide margins along with adjuvant radiotherapy, which leads to better outcomes even in patients with positive surgical margins. Larger clinical trials with Imatinib will firmly establish its chemotherapeutic role in its management. Lifelong regular follow-up is essential for early detection of tumor.