Basaloid squamous cell carcinoma (BSCC) is a rare carcinoma with both basaloid and squamous components. We report a case of BSCC arising in the larynx. A 61-year-old man presented with hoarseness and sore throat. Laryngeal endoscopy revealed a tumor from the left laryngeal vestibule to both vocal cords, which caused paralysis of the left vocal cord. Squamous cell carcinoma (SCC) was confirmed in a biopsy specimen. Additional imaging studies confirmed a diagnosis of laryngeal cancer (supraglottic SCC, T3N2bM0, stage IV A). After preoperative chemotherapy, total laryngectomy and bilateral neck dissection were performed. Histopathological analysis of an operative specimen revealed BSCC, with extracapsular spread in metastatic lymph nodes. Despite adjuvant radiotherapy, multiple lung metastases rapidly developed. Chemotherapy with cisplatin, 5-fluorouracil, and cetuximab was temporarily effective, but metastasis later progressed quickly. BSCC is associated with a poor prognosis and high potential for distant metastasis and thus requires multidisciplinary therapy, including potent chemotherapy.