Cylindric cell carcinomas (transitional cell carcinomas) are a rare and distinct histopathological entity presenting in the head and neck region. They have been known by myriads of nomenclature like cylindric carcinomas, nonkeratinizing sinonasal carcinoma, papillary carcinoma, cylindrical or columnar cell carcinoma, intermediate cell carcinoma, Schneiderian carcinoma, and Ringertz carcinoma. They are considered a variant of nonkeratinizing squamous cell carcinoma. Cylindric carcinomas are usually described in the sinus and nasal cavity and rarely said to involve nasopharynx and larynx. Only passing references have been made for its presentation in oropharynx including tonsils and the base of the tongue. We report here a rare case of transitional cell carcinoma presenting in the base of the tongue. There are no separate treatment recommendations in the literature, and the management is on the lines of treatment of squamous cell carcinoma. We report here a case of cylindric cell carcinoma presenting in the base of the tongue. The patient was staged as having cT2 N3 M0 (Stage IV B) disease. The patient received palliative radiotherapy of 20 Gy in five fractions followed by chemotherapy with injection paclitaxel and carboplatin. A partial response to treatment was achieved at the time of writing this report.