Although basal cell carcinoma (BCC) often occurs in the head and neck region, BCC in the middle ear is extremely rare. As far as we could determine, only five cases of BCC in the middle ear have been reported previously in the literature. We present the case of a 54-year-old Japanese man with BCC in the middle ear. Preoperative histopathologic examination indicated the possibility of skin appendage tumor. Mastoidectomy and extirpation of the lesion were performed, and the resected tumor was finally diagnosed as BCC. Four months after the initial surgery, the patient had local recurrence and reoperation, and postoperative radiotherapy was given. The patient had repeat local recurrence in the parapharyngeal space 3 years and 2 months following postoperative radiotherapy. The patient did not respond to two courses of chemotherapy with cisplatin and 5-fluorouracil, and eventually died of the primary disease 4 years and 7 months after the initial surgical treatment. We reviewed the five cases previously reported in the literature concerning BCC in the middle ear. More case reports concerning BCC in the middle ear are needed to determine its appropriate management.