✓The authors report on a 63-year-old man initially admitted to an outside hospital for altered mental status and respiratory distress. A head computed tomography scan disclosed a right frontal cystic mass, suspected to be a neoplasm. An open biopsy was performed at an outside institution, and on visualization of the cyst, an aneurysm was found incidentally. Postoperatively, an angiogram and magnetic resonance image confirmed the presence of a distal right M1 segment aneurysm. The patient was transferred to our institution where, in addition to the middle cerebral artery lesion, a right anterior choroidal artery aneurysm was found intraoperatively; the necks of both aneurysms were clipped successfully. A review of the literature revealed 14 additional cases of intracranial aneurysms associated with arachnoid cysts. Data in the present report highlight the importance of considering an intracystic aneurysm in the differential diagnosis when reviewing cases that involve a cystic mass with a mural nodule. The authors provide a comprehensive summary of documented cases of aneurysms associated with arachnoid cysts. In addition, they include a discussion of prevailing thoughts on the origin and evolution of arachnoid cysts.