A black teenager presented with long-standing vision loss in his right eye. Clinical exam revealed increased conjunctival vascularity and total exudative retinal detachment of the right eye. Ultrasound imaging demonstrated a choroidal mass with moderate-to-high reflectivity consistent with a choroidal hemangioma. A closer external examination demonstrated a subtle reddish hue of the eyelids and cheek, consistent with a nevus flammeus. MRI of the brain revealed a T2 enhancing vascular mass. Collectively, these findings were consistent with Sturge-Weber Syndrome. This case highlights the need for a high degree of suspicion for Sturge-Weber Syndrome in dark-skinned individuals that can otherwise mask the nevus flammeus. Physical exam findings and radiographic evidence can be used to guide the diagnosis. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:183-186.].
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