Spindle cell carcinoma of the hypopharynx is very rare. A 67-year-old man with spindle cell carcinoma of the pyriform sinus was presented at our clinic. The lesion was large with a massive tumor in the right hypopharynx filling the right pyriform sinus. Computed tomography revealed destruction of the thyroid cartilage. Metastasis was not detected. Since the first endoscopic biopsy of this lesion suggested small cell carcinoma, the patient was treated with chemotherapy consisting of CBDCA and VP-16, and the lesion was markedly reduced. After neo-adjuvant chemotherapy, partial laryngopharyngectomy and right neck dissection were performed. The pathological report for the surgical specimen was spindle cell carcinoma. Adjuvant chemotherapy (same regimen as the neo-adjuvant chemotherapy) was performed. One year after the surgery, a tumorous lesion could be seen in the right tonsil. The lesion was removed and the pathological finding for this lesion was squamous cell carcinoma. The relation between the spindle cell carcinoma of the pyriform sinus and the squamous cell carcinoma of the tonsil was unclear. The patient underwent postoperative radiotherapy at a dose of 42 Gy and shows no evidence of local recurrence or regional metastasis 6 months after the second surgery.