The authors present a case of bilateral painless progressive proptosis. A diagnosis of ocular juvenile xanthogranuloma was made based on clinical manifestations, histopathology, and immunohistochemistrical staining. Genetic testing discovered the BRAF V600E mutation. This patient did not respond to standard chemotherapy; however, he demonstrated regression after anti-BRAF targeted therapy. [J Pediatr Ophthalmol Strabismus. 2021;58(4):e19-e21.].