Encephaloceles are herniation of cranial content arising from a skull defect. Encephaloceles of the lateral wall of the sphenoid sinus (ELWSS) are  uncommon events. In most cases, these cranial hernias are secondary to trauma and craniofacial surgery. Spontaneous forms are evenrarer and not well understood. The most adopted hypothesis is a persisting Sternberg’s canal, an embryonic remnant connecting the middle cranial fossa and the nasopharynx. ELWSS are usually revealed by cerebrospinal fluid (CSF) leak. Diagnosis of this disease necessitates quick management due to the potential of lethal complications such as meningitis. We report the case of a spontaneous ELWSS in a 53-year-old woman revealed by CSF leak which was successfully managed with a conventional transcranial approach. We focus on the clinical aspect and pathogenesis of the disease, and discuss the main possible surgical approaches. Keywords: Spontaneous encephalocele, Sphenoid sinus, CSF leak, Transcranial approach