Spontaneous sphenoidal encephaloceles are rare entities. In the case of intrasphenoidal encephaloceles, most defects are temporosphenoidal and occur in the lateral wall of the sphenoidal sinus. There have been to our knowledge only 7 reports of medial, transsellar encephaloceles in the literature. We report a case of intrasphenoidal transsellar encephalocele that was successfully managed through an endoscopic approach with complementary lumboperitoneal shunting. This disorder presents a challenge in surgical management because of the involvement of opticochiasmatic structures, the hypothalamopituitary axis, and the delicate vasculature of the circle of Willis in and around the encephalocele, besides proximity to the cavernous sinus. Rhinologists should be aware of these malformations, since the optic nerve or chiasma may be totally exposed in the sphenoidal sinus in the course of this disease. This case illustrates the need for magnetic resonance imaging in case of sphenoidal abnormalities, as well as the possibility of endonasal repair without a transcranial approach for large intrasphenoidal encephaloceles.