We report a case of advanced small cell carcinoma in the larynx, which was treated with Irinotecan hydrochloride (CPT-11) chemotherapy. The patient was free of disease for 4 years after treatment. Several chemotherapeutic agents for small cell carcinoma have been proposed; however, median survival time has been miserable, especially in advanced cases. For the cure of the aggressive lethal behavior of this disease, chemotherapy with CPT-11 might be effective to improve median survival of patients with small cell carcinomas of the larynx.