A 78-year-old man who had been diagnosed with autosomal dominant polycystic kidney disease (ADPKD) and hypertension presented with chest pain. His family history was positive for ADPKD. Chest computed tomography (CT) revealed a type A aortic dissection with thrombotic occlusion of a false lumen and an ulcer-like projection in the ascending aorta, an aneurysm of the ascending aorta, and pericardial effusion. Abdominal CT showed multiple renal and hepatic cysts. At surgery, aortic dissection with thrombotic occlusion of the false lumen and an intimal tear in the distal ascending aorta were observed. Hemiarch replacement including the intimal tear was performed. The patient is doing well without requiring dialysis and without recurrence of aortic dissection or aneurysm under strict antihypertensive therapy 3 years after the operation. Pathological examination of aortic wall specimens revealed no degenerative abnormality. ADPKD should be kept in mind as one of the causative disorders of aortic dissection.