Pulmonary vein thrombosis is a rare disease and is usually represented as a complication of pulmonary tumors, lung lobectomy, and atrial fibrillation ablation. Although it is a potentially life-threatening condition, it is easily misdiagnosed because of non-specific symptoms and its characteristics on imaging modalities. Here, we report a case of thrombotic occlusion at the ostium of the left superior pulmonary vein resulting from pulmonary vein stenosis following radiofrequency ablation for atrial fibrillation. This report extends the available approach with anticoagulant therapy and interventional angioplasty to pulmonary vein thrombosis secondary to pulmonary vein stenosis.