Malignant tumors of external auditory canal (EAC) constitute less than 0.2% of all head and neck cancers. The incidence of carcinoma of the EAC is estimated to be between one in six per million populations. Majority of cancers of EAC are squamous cell carcinomas and basal cell carcinomas. Some rare and unusual tumors do occur within the ear canal including malignant melanoma, merkel cell carcinoma, angiosarcoma, lymphoma and adnexal carcinomas like ceruminous adenocaricinoma and adenoid cystic carcinoma. Ceruminous glands tumors constitute about 5% of all external auditory canal tumors. Carcinoma of the external auditory canal is a difficult diagnosis unless the tumors presents as a fungating mass protruding from the external auditory canal. Syringocystadenocarcinoma Papilliferum (SCACP) is an extremely rare cutaneous adnexal neoplasm. Syringocystadenoma papilliferum (SCAP) is thought to be precursor of SCACP. About 50 cases of SCACP have been reported in literature all over the body. The diagnosis is difficult and excisional biopsy becomes mandatory for diagnosis and treatment. We present a case of SCACP in the external auditory canal in a middle-aged female. To the best of our knowledge and belief, this is the first case of SCACP in the external auditory canal in the English literature. This prompted us to report this case.
