A 38-year-old man was found to have an abnormal shadow in his chest X-ray film during a general medical examination. A nodule with a homogeneous density and a clear margin was seen in the upper lobe of the right lung. Since this shadow gradually enlarged during a five-year follow-up period, he was admitted to our hospital. Chest contrast and dynamic CT scans and aortography showed characteristics of a benign tumor with somewhat rich vascularity. The doubling time of this tumor was about 430 days. Transbronchial brushing for cytologic study and a transbronchial biopsy showed no malignancy. However, the tumor was surgically resected because of its gradual growth over the five years. The resected tumor was histologically diagnosed as a benign clear cell tumor of the lung. This disease was first described by Leibow and Castleman in 1963, as a benign pulmonary tumor similar to metastatic renal cell carcinoma. This disease is extremely rare and only 61 cases have been reported: twenty cases, including the present case, in Japan and 41 in other countries.