Autosomal dominant polycystic kidney disease(ADPKD) is one of the most common hereditary diseases, and frequently has well defined extrarenal manifestations. Among the fatal vascular complications associated with ADPKD, ruptured intracerebral aneurysm and ruptured abdominal aortic aneurysm are widely known. Intramural hematoma(IMH) is a rare but potentially disastrous complication, and clinicians should always consider this when dealing with patients with ADPKD who present with acute dyspnea or chest pain. We report a case of a 67-year-old woman with the IMH as a fatal complication of ADPKD and intracranial aneurysm.