Introduction: Inverted papilloma of the head and neck is a locally aggressive benign epithelial tumor. In most cases the tumor arises from the lateral wall of the nasal cavity involving both the nasal cavity and maxillary and ethmoid sinuses. Isolated inverted papilloma of the sphenoid sinus is exceedingly rare. Objective and Case Report: To relate a case of isolated inverted papilloma of the sphenoid sinus that was completed excised by endoscopic ethmoidectomy and sphenoidectomy, discussing its diagnose and treatment. Conclusion: Inverted papilloma should be considered as a differential diagnose of mass isolated to the sphenoid sinus. Clinical commom presentation is headache, visual disturbances and other cranial nerve disfunctions. Imaging exams and histologic findings are essencials to the diagnose. Functional endoscopic sinus surgery is an efective option in the treatment of this lesion.