Here, we report a very rare case of spontaneous intraventricular tension pneumocephalus. This case concerns a 40-year-old patient with medical history of a tumor of the pineal region and a secondary hydrocephalus treated by multiple ventriculoperitoneal shunts. He presented in the emergency room because of unusual headaches, nausea, and visual loss. In addition, he reported slight rhinorrhea in the past few weeks. The initial brain computed tomography scan revealed a spontaneous intraventricular tension pneumocephalus. There was no history of recent head trauma and no sign of disconnection of the shunt system. A complementary radiologic assessment including a thin-slice bone computed tomography scan and a radioisotope cisternography revealed an osseous defect and an isotope leakage at the junction between the tegmen tympani and the squamous part of the left temporal bone. A middle cranial fossa surgery was performed to repair the osteo-meningeal breach.