Objective: To present a case of a fourth ventricle subependymoma (SE) with a spontaneous acute subarachnoid intra-cisternal bleeding. Methods: A 33-year-old man was admitted with 5 days history of oppressive occipital headache and neck pain without additional neurological focus. Unenhanced computed tomography (CT) scan demonstrated an isointense mass located in the fourth ventricle with a spontaneously hyperdense acute extratumoral hemorrhage in the cisterna magna. Contrast-enhanced magnetic resonance imaging (MRI) revealed a well-delimitated non-enhanced tumor, hypointense on T₁-weighted and hyperintense on T₂-weighted images, involving the floor of the fourth ventricle and extending caudally into the cervical spinal canal via foramen magnum. Results: Intraoperative, a large blood clot was removed and a macroscopically hypovascularlesion was completely excised from the right lateral recess and the floor of the fourth ventricle. Intra and postoperative immuno-histopathological examination revealed a SE. The patient has a normal postoperative course and was discharged in the fifth postoperative day. A 10-month postoperative MRI study confirmed a complete tumor resection. Conclusion: Symptomatic SEs should be surgically treated emphasizing the urgency in the presence of hemorrhage. The interest of this case is to demonstrate that infratentorial SEs although extremely rare, might present with acute subarachnoid bleeding.