A 38-year-old woman had complex, congenital heart anomalies, including double-outlet right ventricle with mitral atresia, severe left ventricle hypoplasia, atrial septal defect, and severe pulmonary stenosis, for which she had not received appropriate treatment. She presented to our institution with an intense sudden-onset headache that had initiated while sleeping. Brain computed tomography indicated the presence of spontaneous subarachnoid hemorrhage. Cerebral angiography revealed a left middle cerebral artery aneurysm. Two days later, she underwent a craniotomy for aneurysm clipping. According to the pathological analysis, culture, and clinical presentation, the aneurysm was not mycotic. The patient regained consciousness after a few days postoperatively. Despite the complex and rare congenital heart disease, it is especially notable that the patient survived 38 years without treatment and later had a ruptured cerebral aneurysm that was successfully managed operatively. Our literature review indicated that some congenital heart diseases such as aortic coarctation may correlate with the presence of intracranial aneurysms. Our case is novel because there are no similar cases of complex congenital heart disease combined with a ruptured intracranial aneurysm successfully resolved operatively. We believe that this case would help clinicians deal with such complicated cases of congenital heart disease combined with intracranial aneurysms.