We describe the case of an 8-year-old girl without neurofibromatosis, who presented with total loss of vision on the left eye, due to a chiasmatic mass with imaging characteristics of glioma, accompanied by a second asymptomatic mass in the middle cranial fossa, along the intracranial route of the right trigeminal nerve. The patient received a total of 10 weekly injections of vincristine and four injections of carboplatin every 3 weeks and achieved a very good partial response (97% volume reduction) after the nineth week of therapy with acceptable toxicity. Given the natural history of opticochiasmatic gliomas, we cannot rule out the possibility of a spontaneous regression. However, we believe the quick response accompanied by visual improvement was most likely due to chemotherapy. A trial of vincristine and carboplatin may be worthwhile in children with symptomatic chiasmatic gliomas, irrespective of their age.