A 6-year-old male presented with a large, fleshy mass protruding from the left eye that has been rapidly enlarging for 4 weeks before presentation (Fig. 1A). Since infancy, he manifested with mental retardation, right hemiparesis, motor seizures, and he underwent surgical correction of an undescended testicle. His photograph at the age of 10 months revealed left eye esotropia, hazy cornea, and a flat red lesion on the temporal conjunctiva (Fig. 1B). On examination, the patient exhibited frontoparietal alopecia, faint scalp nevus, multiple eyelid and periocular skin tags, and dental anomalies.