We report a case of coexistence of sarcoidosis and thymic carcinoid. A 57-year-old man was pointed out the anterior mediastinal tumor when his generator of pacemaker was exchanged. The tumor was diagnosed as atypical carcinoid by percutaneous needle biopsy. Chest computed tomography (CT) revealed the mediastinal and right hilar lymphadenopathy. Preoperative transbronchial aspiration cytology revealed no malignancy and extirpation of the anterior mediastinal tumor was carried out together with left diagraphmatic nerve and pericardium. The histopathological examination of the pretracheal lymph node was sarcoidosis. Postoperative radiation was performed because the thymic carcinoid invaded the pericardium. The right hilar lymph node was enlarged after the radiation. Five months later, follow-up chest CT showed reduction of the right lymph node. He has been alive without recurrence of the thymic carcinoid for 3 years. Simultaneous occurrence of sarcoidosis and thymic carcinoid is extremely rare. Assessment of mediastinal lymph node is difficult either preoperatively and postoperatively. Histological confirmation of the lymph node and careful follow-up are necessary.