Purpose To describe cases of optic pathway glioma (OPG) warranting treatment in children. Methods This is a retrospective review of pediatric patients treated for OPG. The clinical data and imaging studies were obtained from the medical records and radiology files of patients seen at the Pediatric NeuroOphthalmology Clinic at the University of Illinois, Chicago and the private office of the author (LMK). Results A total of seven cases with an age range of 3-48 months at presentation were reviewed. Three of the patients were also ultimately diagnosed with neurofibromatosis type 1. Presenting symptoms included proptosis, decreased vision, gaze deficit, and nystagmus. Four patients underwent biopsies that confirmed OPG. Six of the patients were treated with intravenous chemotherapy, with three patients requiring a second chemotherapy cycle. One patient was successfully treated with an en-bloc optic nerve excision. Two patients underwent unilateral enucleation owing to globe complications. Conclusion Although benign tumours, OPG can behave very aggressively in young children. Both chemotherapy and en-bloc excision can be employed for treatment.