Tehran University of Medical Sciences (TUMS) Academic Journal of Surgery 2423-3218 7 3 2024 10 30 72 75 Abolfazl Taheri 1 Otorhinolaryngology, Head and Neck Surgery, School of Medicine, Baqiyatallah University of Medical Sciences, Tehran, Iran. 2 New Hearing Technologies Research Center, Clinical Sciences Institute, Baqiyatallah University of Medical Sciences Tehran, Iran. Yaser Ghoroghchiyan 1 Otorhinolaryngology, Head and Neck Surgery, School of Medicine, Baqiyatallah University of Medical Sciences, Tehran, Iran. 2 New Hearing Technologies Research Center, Clinical Sciences Institute, Baqiyatallah University of Medical Sciences Tehran, Iran. 3 Student Research Committee, Baqiyatallah University of Medical Sciences, Tehran, Iran. Parisa Mohsenzade Departments of Orthodontics, School of Dentistry, Shahid Sadoughi University of Medical Sciences, Yazd, Iran 2024 07 14 Introduction: Dermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive tumor with high rate of local recurrence but low metastatic potential. Its occurrence in the head and neck is a rare finding but it is associated with increased morbidity and mortality. Case report: A 53-year-old Iranian man referred to a complaint about an increase in the size of the subcutaneous mass on the right cheek that had been present since he was 17 years old. On clinical examination, the lesion was not painful and had no erythema. In examining the patient's CT scan without contrast, a mass-like area with soft tissue density was seen. During the surgery, the flap was lifted after a lateral rhinotomy incision. The biopsy sample was sent for frozen section, and the pathology result was a Spindle cell tumor. In immunohistochemistry (IHC), two factors, including CD34 and VIMENTIN, were positive and Dermatofibrosarcoma protuberans (DFSP) was reported. Conclusion: The most important prognostic factor for surgery of Dermatofibrosarcoma protuberans is obtaining tumor free surgical margins. As a result, accurate diagnosis and correct management of this uncommon and locally invasive malignancy is very important, especially in head and neck surgery. https://ajs.tums.ac.ir/index.php/ajs/article/view/267