Vol. 7, Issue 2, Part A (2025)

Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder characterized by facial port-wine stains, leptomeningeal angiomatosis, and ocular abnormalities. We present a 7-year-old girl with intractable seizures whose neuroimaging revealed classic features of SWS along with an unusual finding of crossed cerebellar diaschisis. Initial CT demonstrated right parieto-occipital and frontal tram-track calcifications with cerebral volume loss. MRI revealed right cerebral leptomeningeal angiomatosis with prominent medullary veins, choroid plexus enlargement, and notably, left cerebellar volume loss with signal abnormalities consistent with crossed cerebellar diaschisis. This finding, rarely reported in SWS, likely represents functional disconnection between the affected cerebral cortex and contralateral cerebellum due to chronic hypoperfusion. While infratentorial involvement in SWS is increasingly recognized, crossed cerebellar diaschisis remains an exceptional finding that may indicate more severe hemispheric dysfunction. Recognition of this phenomenon expands our understanding of SWS's neurological impact and may have prognostic implications for patient management