A five-year-old girl with Sturge-Weber syndrome (SWS) presented with unilateral leukocoria. Imaging excluded retinoblastoma and revealed a choroidal hemangioma, a non-traumatic vitreous hemorrhage and a cataract. To the best of our knowledge, this is the first case report in the radiologic literature of these ophthalmologic findings presenting together and conditioning leukocoria in the setting of SWS. We discuss possible mechanisms and review the literature, emphasizing the role of neuroimaging in pediatric patients with SWS and ophthalmologic complaints.
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