We present an unusual case of an epidermoid cyst in the sphenoid sinus with extensive bony erosions and mass effect, compressing the optic nerve causing visual disturbances. Epidermoid cysts of the paranasal sinuses are extremely rare cases. They usually present as intradural, extraaxial, benign masses of the cerebellopontine angle but they should also be considered in the differential diagnosis of sphenoid sinus lesions. The typical imaging appearance of craniocerebral epidermoid cysts is demonstrated and pathophysiological considerations of formation in the paranasal sinuses are discusse