━━ Background. Hilar and/or mediastinal lymph node (LN) cancer without a primary tumor is rare. Case. A 50-year-old man underwent distal gastrectomy for gastric cancer in year X. Although preoperative fluorodeoxyglucose (FDG)-positron emission tomography (PET) showed an LN of 11 mm in diameter with the abnormal uptake of FDG in the subaortic region (LN#5), a biopsy was not requested. In year X+1, LN#5 shrank to 3 mm, and the carcinoembryonic antigen (CEA) concentration decreased from 56.6 ng/ml to 8.6 ng/ml after gastric cancer surgery. However, the mediastinal LN slowly enlarged from year X+2, increasing to 24 mm at year X+ 11; the CEA concentration increased to 1760.4 ng/ml and a nodule appeared in the S 1+2 segment of the left lung. Surgery was performed. Thoracoscopic resection of LN # 5 and left upper division segmentectomy was performed. The postoperative pathological diagnosis was adenocarcinoma in both the pulmonary nodule and LN#5, and immunostaining was positive for thyroid transcription factor-1 and napsin A. Eleven years later, the primary lesion appeared in the lung and was diagnosed as lung cancer and mediastinal LN metastasis, pT1bN2M0 stage IIIA. Conclusion. Patients with lymph node carcinoma of unknown origin may develop a primary lesion after a long period of time. This case is instructive in suggesting the importance of long-term follow-up in such cases.