Pulmonary arteriovenous malformation (PAVM) consists of abnormal communications between the pulmonary arteries and veins. Because PAVM may cause neurological defects (such as stroke and brain abscess) or hemoptysis and hemothorax, embolization therapy or surgery is required. Resection using video-assisted thoracic surgery (VATS) has recently been performed for small peripheral PAVMs. For large or hilar PAVM, surgical resection with thoracotomy is required. We report herein a case of PAVM (4.0 × 3.5 cm) located in the left S6 segment near the lung hilum that was resected successfully using simultaneously stapled S6 segmentectomy (simultaneous stapling of anomalous vessels of the PAVM and hillar structures of S6) with VATS.