Purpose Sebaceous carcinoma (SC) is a rare adnexal tumor. Extra-ocular, facial SC is very uncommon and local metastases are an extreme rare finding. A respective case is presented and discussed together with the current literature. Case and Review A tumor of the left ear of an 87-old male was primary excised together with multiple suspicious lesions of the head and neck. Most specimens were histopathologically rated as squamous cell carcinomas (SCC). Despite the in-sano resection, additional new suspicious retro-auricular and temporal lesions were detected. Successive resections were diagnosed as basal cell carcinomas (BCC) and, because of a non-in-sano resection in a third approach, as SC. After reappraisal and immunhistochemical staining [epithelial membrane antigen (EMA), CK 5-6 and CD 15], most of the former specimens turned out to be SC as well. A literature search showed 3 reported cases of extra-ocular head and neck SC with cutaneous local metastases. In another review, in a total of 168 cases, SC was diagnosed after wrong initial histological diagnosis (SCC n = 56, BCC n = 44; other entity or precursors of carcinomas n = 68).Conclusion Due to inconsistent histologic patterns, histopathological misdiagnosis of the uncommon facial SC and its metastases may complicate further therapy, prolong treatment and may lead to a worse prognosis of this neoplasm. A close interdisciplinary collaboration of clinician, surgeon and pathologist is of most relevance for the right diagnosis.