We present a case report of a 72-year-old man with an anomalous lobar pulmonary vein which was initially misdiagnosed as a pulmonary arteriovenous malformation on chest computed tomography. This uncommon condition was correctly diagnosed during pulmonary angiography performed as workup for the computed tomography finding. In this report, we describe the imaging findings in this case and discuss congenital anomalies of the pulmonary veins.