Abstract: Unilateral absence of the pulmonary vein (UAPV) is a rare congenital anomaly, and computed tomography (CT) angiography can clearly show the anatomical morphology of the pulmonary artery, pulmonary vein, and related lesions. Here, we report the case of an asymptomatic 28-year-old female patient with unilateral interstitial changes in the lungs on plain CT scanning due to UAPV, diagnosed on CT angiography, with no abnormalities in her laboratory tests. This report summarizes and analyzes the imaging manifestations of UAPV in conjunction with related literature to improve our understanding and the diagnosis of this rare disease.