We report here a long-term survival case of advanced esophageal cancer with distant lymph node metastases, treated with definitive chemoradiotherapy (CRT). A man in his 60s with disturbance of swallowing was diagnosed as middle esophageal cancer involving multiple metastases of distant lymph nodes. CRT (combination of 5-FU and nedaplatin every four weeks for four courses with 66 Gy of radiation) was administered. After a completion of CRT, CT scan revealed shrinking metastatic lymph nodes. No tumor but a scar at the site of cancer was observed by endoscopy, and histopathology of biopsy specimen detected no tumor cells. From these results, we diagnosed the curative effect of CRT as complete response. Five years after CRT, a swelling of left inguinal lymph node with uptake of fluorine-18-fluorodeoxyglucose appeared and was extirpated, and the swelling was diagnosed histopathologically as metastasis of esophageal cancer. The patient is surviving with no recurrence for 7 years and 8 months from the first diagnosis. In cases of highly advanced esophageal cancer, a long-term follow-up should be performed.