Our patient was born at term with buphthalmos and proptosis of her right eye, accompanied by corneal clouding and increased tearing. She was subsequently noted to have right sphenoid wing dysplasia, multiple cutaneous plexiform neuromas of the right eyelids and face (fifth nerve distribution), right hemispheric dysplastic polymicrogyria, and a seizure disorder. She was diagnosed as having NF1. The left eye was within normal limits. The patient received a Baerveldt glaucoma drainage implant at age 5.5 months. At age 8 months, a dilated fundus examination revealed temporal retinal whitening and posterior retinal hemorrhages, which were initially attributed to a retinal vein occlusion. Four months later, the patient was diagnosed clinically as having a CHRRPE (Figure 1). Enlargement of the retinal tumor was noted over the following 8 months, accompanied by the development of a vitreous hemorrhage, tractional retinal detachment, and proliferative vitreoretinopathy. At age 20 months, the right eye was enucleated owing to it being blind and painful with a fixed pupil and corneal haze. Postoperatively, she was more comfortable, no longer photophobic, and without tearing.