Introduction. Ectopic sphenoid sinus pituitary adenomas are a rare, benign pituitary tissue neoplasm, occurring separate from and without involvement of the anterior pituitary gland. We present a case of ectopic pituitary adenoma confined to the sphenoid sinus, asociated with an empty sella.Case report. A 52-year-old man was addmitted to our neurosurgical department with persistent headache and nasal obstruction of one year duration. Patient presented no endocrine abnormalities and no visual disturbances. MRI revealed a well-defined mass lesion in the sphenoid sinus, hypointense on T 1 -weighted images, hyperintense on T 2 -weighted images, with moderate heterogeneous gadoliniumenhancement. A primary empty sella was associated. Surgery was performed by transnasal transsphenoidal approach. The tumor was removed in a piecemeal fashion. The bony sellar floor was intraoperatively proven to be intact. The pathologic studies confirmed the diagnosis of ectopic nonfunctional pituitary adenoma of the sphenoid sinus. The postoperative course was uneventful.Conclusions. When encountering a tumor within the sphenoid sinus, an ectopic pituitary adenoma should be suspected.A clinical, imaging and endocrinologic evaluation must be undertaken, aiming to identify the nature of the lesion and to initiate the specific therapy. Surgery remains the only way of obtaining a definite diagnosis and cure.