Thymolipoma is extremely uncommon benign mediastinal tumor consisting of fatty and thymic tissue. Only 50 verified cases have been reported in the world literatures. This one case is the first reported example of surgically treated thymolipoma associated with spontaneous pneumothorax. A thirty-two year old male patient had been in good health until two days prior to admission, when he noted sudden dyspnea associated with an aching pain over the left precordium. The dyspnea and chest pain had become progressively worse. The physical examination revealed that left hemithorax was tympanic sound on percussion and absence of breathing sound on auscultation and point of maximal impulse was located on the 4th intercostal space at the left sternal border. Emergency closed thoracostomy was performed under the impression of tension type spontaneous pneumothorax of the left lung. After closed thoracostomy, point of maximal impulse was not changed inspire of full expansion of the left lung and chest X-ray was strongly suggested pericardial effusion or cardiomegaly which couldn`t account for by clinical course and hemodynamic evidence. EKG, echocardiogram, bronchofiberoscophy, bronchogram and diagnostic thoracentesis was performed. On Dec. 8, 1980, operation was performed under the impression of mediastinal tumor in the anterior mediastinum. At left posterolateral thoractomy, a large fatty mass, measuring 35 x 27 x 13 Cm in dimension and weighing 3350 gm, was resected and multiple bullae on the apicoposterior segment of the left upper lobe was resected and continuously sutured. The pathologic diagnosis of the fatty mass was thymolipoma. The postoperative course was uneventful and discharged in good general conditions.