Most hiatal hernias present in patients older than 40 years, and the incidence increases with age. The para-esophageal type is rare but it can be potentially life threatening because of the risk of volvulus and incarceration. We present a 73-year-old woman who presented with chest discomfort, dyspepsia, and regurgitation for more than two weeks. In posteroanterior chest radiography showed serpiginous lesion on the left lower lung. The computed tomo-graphic scan with contrast enhancement also showed a tortuous vascularity lesion in the laterobasal segment of left lower lung and herniated stomach behind the heart. In the purpose of management of combined hiatal hernia and pulmonary arteriovenous malformation, a Belsey mark IV antireflux procedure and a wedge resection of the left lower lobe via a left posterolateral thoracotomy were performed. On the ninth post-operative day, she was discharged and recovered very well, and followed up in our clinic.