Congenital pulmonary vein stenosis (PVS) is a very rare cardiac malformation and commonly associated with cardiac or extracardiac abnormalities. It is usually found during the newborn period and survival to maturity is very rare due to either of progressive pulmonary hypertension or associated cardiac anomalies. In this case report, an adult patient with congenital PVS that was incidentally found during an evaluation for pneumonia, was treated with balloon angioplasty. After balloon angioplasty, the pressure gradient between the stenotic pulmonary vein and left atrium was significantly reduced and this patient had a benign course during the routine follow-up. Although there is no consensus concerning the optimal treatment strategy for this anomaly, balloon angioplasty can be a reasonable therapeutic option for the palliation of adult congenital PVS.