Arachnoid cysts (AC) are intraarachnoidal cerebrospinal fluid collections, and account for 1% of all intracranial space-occupying lesions. Intracystic hemorrhage of the AC can occur spontaneously, but this is an extremely rare event. Herein, we present a case of hemorrhagic AC in a nontraumatic patient in the left middle cranial fossa. We also performed relevant literature review on this disease.Arachnoid cysts (AC) are intraarachnoidal cerebrospinal fluid collections, and account for 1% of all intracranial space-occupying lesions. Intracystic hemorrhage of the AC can occur spontaneously, but this is an extremely rare event. Herein, we present a case of hemorrhagic AC in a nontraumatic patient in the left middle cranial fossa. We also performed relevant literature review on this disease.