Tumors originating from ceruminous glands are rare lesions of the external auditory canal. The lack of specific clinical and radiological signs makes their diagnosis challenging. We report the case of an exceptionally rare benign tumor, a syringocystadenoma papilliferum (SCAP), in an atypical location in the bony segment of the external auditory canal with uncommon clinical signs. The special traits of the case included the following: the most lateral component of the tumor was macroscopically cystic and a granular myringitis with an obstructing keratin mass plug was observed behind the mass. The clinical, audiological, radiological, and histological characteristics of the neoplasm are consequently presented. Intraoperative diagnosis of the epidermal cyst was proposed. The final diagnosis of SCAP was determined only by histological analysis after the surgical excision. The educational aspects of the case are critically discussed.