A 56-year-old female patient presented with history of nasal blockage, nasal bleeding, headache, since one month. On admission the patient developed severe headache, decreased vision of eyes and blindness. Clinical diagnosis of rhino-orbital involvement was confirmed by computerized tomography of the paranasal sinuses. The diagnosis of fungal infection was confirmed by KOH examination, haematoxylin and eosin examination and Gomori's methanamine silver stain of the biopsy material. Diagnosis was confirmed by culture on Sabouraud Dextrose agar, slide culture on Czapek Dox medium and the isolate was identified as Saksenaea vasiformis. The patient was started on intravenous amphotericin B (received only one dose before succumbing), but she did not respond to the treatment, developed hemiparesis, slurred speech, diminished reflexes and ultimately died. The involvement of the brain was confirmed by computerised tomographic scan. We believe this case to be the first case of rhino-orbito-cerebral infection due to Saksenaea vasiformis and that of second case in an immunocompetent patient in the world.