This is the first case report of atypical fibroxanthoma in the otolaryngology literature. Atypical fibroxanthoma is a rare fibrohistiocytic tumor that occurs on actinically damaged skin. The typical presentation is a nodular lesion in sun-exposed areas of the head and neck in an elderly patient. Differentiating atypical fibroxanthoma from other soft tissue sarcomas, such as dermatofibrosarcoma protuberans and malignant fibrous histiocytoma, can be difficult. Treatment should be wide local excision with a 1-cm margin. In cosmetically important regions in which tissue preservation is important, Mohs' surgery should be considered.