This report describes the case of a chondroid syringoma occurring in the nasofacial groove of a 60-year-old woman. This benign, mixed epithelial tumor is infrequently seen by the otolaryngologist-head and neck surgeon, and therefore may not be included in the differential diagnosis of a nodular lesion on the skin of the head and neck. However, the most frequent site of occurrence for these relatively rare cutaneous lesions is the head and neck region. Histologically, these tumors are quite similar to pleomorphic adenomas of salivary gland origin, and optimal surgical management similarly requires removal of a cuff of normal tissue, rather than a simple shelling out of the lesion. The clinical presentation, pathology and recommended management of this rare tumor are discussed.