Sturge–Weber syndrome (SWS) is a rare congenital neurocutaneous disorder characterized by facial port-wine stain, leptomeningeal hemangiomas, and ocular manifestations such as glaucoma and diffuse choroidal hemangioma (DCH). DCH is a benign vascular lesion characteristically seen in up to 50% of patients affected with SWS. Diffuse choroidal hemangioma can cause refractive error, foveal distortion, and exudative retinal detachment and contribute to vision loss. We report a case of a young male with SWS and a unilateral DCH with characteristic imaging features.