A 51-year-old man had been diagnosed with esophageal achalasia 26 years previously; however, regular medical examination was not performed. He presented to the hospital for severe back pain and increased dysphagia during the past 7 months. Endoscopic examination showed the presence of multiple primary carcinoma of the esophagus with achalasia. An esophagogram showed sigmoid-type achalasia with grade III dilatation. Subtotal esophagectomy with mediastinal and abdominal lymphadenectomy was performed. Histopathological examination showed three squamous cell carcinomas at pStage IVB, including pT3 and pM1b. Furthermore, all the three lesions located in the large, slightly iodine-stained area showed hyperplasia with inflammatory cells. Although adjuvant chemotherapy was administered, the patient died of recurrence on the right chest wall and in the stomach, liver, and left iliac bone at 23 months after operation. A patient with esophageal achalasia should be carefully followed up by endoscopy with iodine staining.