Clear cell adenocarcinoma is an extremely rare tumor of the head and neck region. We report a case of a 75-year-old Caucasian woman with a 10-day history of hemoptysis but with no pain or other significant symptoms. A head and neck computed tomography scan with contrast medium showed an irregular, soft-tissue-like, irregularly enhanced lesion of the base of the tongue extending to its posterolateral portion. The tumor reached the lateral wall of the oropharynx, which showed a nonhomogeneous aspect. The patient underwent resection of the tumor via a conservative transmandibular approach. A clear cell adenocarcinoma of the base of the tongue is rarely a primary malignant lesion; it is more frequently a secondary lesion from a metastatic renal tumor. Because of this neoplasm's relatively slow growth rate and low incidence of metastasis or local recurrence, the gold standard of treatment is complete excision of the tumor with a sufficient tumor-free margin.