We report a patient with an absent right lobe of the lung, a very rare anomaly. A superior lobectomy was performed for adenocarcinoma, and the right upper bronchus and pulmonary artery of the upper lobe (truncus superior) were absent. The first artery originating from the right main pulmonary artery was located at the major fissure, and four relatively small arteries entered the superior lobe. Anatomically, in this patient, the superior lobe was determined to be the middle lobe. The natural upper lobe was missing.