One type of congenital intrasphenoidal meningoencephalocele is remnant of lateral craniopharyngeal (Sternberg's) canal. We present a case of a 23-year girl with 10-month history of right side CSF rhinorrhea. CT scan, MRI revealed congenital meningoencephalocele and CSF leak from middle fossa to right side of sphenoid sinus, and there were bony defects at the floor of the anterior aspect of the right middle fossa. Transcranial repair was performed with right side pterional craniotomy. Careful preoperative evaluation and localization of the sphenoid defect are essential for selection of the best possible surgical approach and skull base reconstruction for repair of sphenoid sinus CSF leaks and meningoencephaloceles. In this case, an endoscopic technique was not successful so, transcranial repair was performed with right side pterional craniotomy.