We report a case of an esophageal collision tumor composed of squamous cell carcinoma and small cell carcinoma (SmCC). A 66-year-old man complained of chest pain after oral intake for nearly one month. The patient received two cycles of neoadjuvant platinum-based combination chemotherapy and enhanced computed tomography showed a partial response of the tumor. He then underwent a thoracolaparoscopic esophagectomy with extensive mediastinal lymphadenectomy. Two cycles of chemotherapy and prophylactic irradiation of the lymphatic drainage region were sequentially achieved after surgery. The patient has survived for more than 18 months with no evidence of recurrent disease since surgical resection.