Symptomatic Rathke cleft cysts (RCCs) are rare sellar and suprasellar lesions, and apoplexy is one of the most unusual presentations. Only a few cases of hemorrhagic apoplexy of RCCs have been reported and their pathogenesis is still poorly understood. In order to present a diagnostic thread to reduce misdiagnosis rate preoperatively, we reported one case of RCC apoplexy and reviewed the associated published literature. we also summarized the clinicopathological relationship on clinical symptoms, imaging features and intraoperative visualization of intracystic content.