Four cases of sphenoid sinus carcinoma have been observed for last 10 years and we reviewed English literatures about sphenoid sinus carcinoma. The sphenoid sinus carcinoma is rare and the diagnosis is difficult. In the early stage, the non-specific deep constant headache is the only symptom but if the sinus wall is penetrated, the neuro-ophthalmologic symptoms and signs may appear. The extension of lesion is identified by radiologic imaging and the diagnosis requires direct biopsy. In case of deep constant headache combined with neuro-ophthalmologic symptoms and signs the sphenoid sinus carcinoma should be considered. Our small data reveals that the radiation treatment offers a possibility of relatively good outcome, although most of the cases are advanced already on initial diagnosis.