Mucoceles are expansile, cyst-like lesions, produced when the natural ostium of a sinus becomes occluded, ratairing the mucus in the sinus itself. Sphenoid sinus mucoceles arise in only about 1% of these mucoceles and are very rare. Signs and symptoms are different depending on multiple important contiguous neurologic and vascular structures. Radiologic evaluation helps in correct assessment, and treatment should include surgical drainage and removal. The authors have experienced a case of sphenoid sinus mucocele which was removed by intranasal sphenoethmoidectomy.