We report a case of anomalous continuation of the inferior vena cava with azygos vein, defect of left pericardium, and dysgenesis of lobes of the lungs. A 43-year-old man came to our hospital, complaining of dyspnea and cough with blood in the sputum. The chest roentgenogram showed moderate cardiomegaly with deviation to the left side of the thorax. Therapy with diuretics and bronchodilators reduced his complaints. A bronchogram and fiberoptic bronchoscopy showed double bronchial branches to the right upper lobe, one from the trachea and the other from the right main bronchus, and a blind pouch of the left lobe. Thoracic computed tomography showed the heart just behind the sternum. Venography showed anomalous continuation of the inferior vena cava and dilated azygos vein. We could not find such a complicated anomaly of the thoracic vessels and bronchus in an adult in the literature, so we believe that it is important to reconfirm the position of major vessels or organs before a thoracic procedure.