A 74-year-old woman visited our hospital with a chief complaint of anterior thoracic discomfort while eating. Esophagogastroduodenoscopy (EGD) revealed two type 2 neoplastic lesions in the 24 cm and 32 cm incisors. Squamous cell carcinoma was observed on histopathological examination. Contrast-enhanced CT showed lymph node and liver metastases, and cStage 4b (T3N3M1) was diagnosed. Ten courses of pembrolizumab plus chemotherapy were administered. Hepatotoxicity developed as an immune-related adverse event after the 10 courses but was resolved with prednisolone (1 mg/kg). Contrast-enhanced CT showed shrinkage of the lymph nodes and disappearance of the liver metastases. Scarring of both esophageal lesions appeared on EGD, and no cancer was detected. The response to the chemotherapy was considered to be complete. We report a rare case of complete response in a patient with unresectable esophageal cancer.