AbstractSebaceous carcinoma is a rare cutaneous malignant neoplasm with an estimated incidence rate of approximately 1–2 per 1 000 000 per year. It rarely presents as an extra-ocular type and it affects patients in between the sixth and eighth decades of life. This is a case of a 31-year-old male with a 10-year history of a right axillary mass with an incisional biopsy of a sebaceous carcinoma. The mass was 10 × 6 cm in largest diameter, erythematous, non-tender and irregularly shaped. The patient underwent wide excision of the right axillary mass with negative margins confirmed by frozen section under general anesthesia. A parascapular fasciocutaneous flap was done in order to provide coverage for the defect. The patient was then discharged improved on the fourth postoperative day. Although there has been no established clinical protocol for the staging, medical and surgical management for extra-ocular sebaceous carcinoma, early diagnosis accompanied with the proper surgical intervention, such as oncologic wide excision with negative margins, were both adequate and paramount to the diagnosis, course and treatment of this patient, given its rarity.