Sphenoid meningoencephaloceles are rare, and their treatment is challenging. In this report, we describe two clinical cases of sphenoid meningoencephalocele, in which one patient presented with a cerebrospinal fluid leak after repeated head trauma, while in the other, sphenoid meningoencephalocele was detected during the study of memory impairment as the patient was otherwise asymptomatic. The CT scans showed bony dehiscence on the lateral wall of the sphenoid sinus filled with soft tissue that was confirmed by MRI as being herniated brain tissue. A transpterygoid endoscopic endonasal approach was performed with a multilayer reconstruction of the defect with success in both cases without perioperative complications. Imaging techniques are fundamental for diagnosis and surgical planning. Treatment using an endoscopic endonasal approach is efficient with very low morbidity.