BACKGROUND: Ceruminous glands are modified apocrine glands, situated in the external auditory canal, that, together with sebaceous glands, produce cerumen, better known as ear wax. The neoplastic transformation of these structures is very rare and there have been few cases reported in the literature.

CASE REPORT: Syringocystadenoma papilliferum is one of the rarest tumors among benign tumors arising from the ceruminous glands. We here report the case of a 72-year-old man with a lesion histologically documented as a syringocystadenoma papilliferum and we review the literature, focusing our attention on clinical features and treatment options of benign glandular tumors arising from the external auditory canal.

CONCLUSIONS: Syringocystadenoma papilliferum is a rare benign tumor of the ceruminous glands of the external ear canal. Excision biopsy is mandatory for the diagnosis and is the best treatment.

Keywords: Adenoma, Pleomorphic, Cystadenoma, Serous, Ear Canal