Primary sphenoid sinus cancer is a rare malignacy. The early symptoms were always subclinically and most of the cases were found to have locally advanced invasion before treatment. We identified a male with presentation of headache and blurred vision for 2 months. Physical examination revealed neuro-ophthalmologic symptoms of left eye. CAT scan disclosed a contrast enhanced soft tissue mass occupied the sphenoid sinus with invasion to left side of pterygopalatine fossa, posterior ethmoid sinus, optic canal, posterior orbital fossa and cavernous sinus. It was biopsy-proven undifferentiated carcinoma. Split-course radiotherapy with a total tumor dose of 7000 cGy in 42 fractions was given to tumor bed over a period of 66 days. Split for 11 days was done when tumor dose reached 4000 cGy. Nearly total regression of primary tumor which was identified from CAT scan and marked improvement of the symptoms were noted at the end of irradiation. But metastases over left submandibular and multiple skeletal regions developed 3 months after the completion of radiotherapy. He died of disease 7 months later. The interval from diagnosis to death was just for 12 months.