VPS13B is a conserved lipid transport protein that mediates transfer of lipids between organellar membranes at contact sites 1. The protein localizes to multiple cellular compartments including the Golgi apparatus, where it functions at interfaces between Golgi cisternae 2, and at ER exit site-Golgi interfaces where it interacts with Sec23IP to promote tubular ERGIC formation 3. VPS13B serves as a tethering factor in endocytic recycling pathways and is essential for Golgi organization and reformation 2. Recently, VPS13B has been shown to recruit phosphatidylinositol-4-phosphate-rich vesicles to mitochondrial fission sites, where it facilitates proper mitochondrial morphology and quality control 4. VPS13B mutations cause Cohen syndrome, an autosomal recessive developmental disorder characterized by microcephaly, intellectual disability, developmental delay, and hypotonia 5. Neuroanatomical changes in VPS13B-deficient mice include reduced dentate gyrus size and motor cortex thinning, suggesting increased neuronal death during infancy 5. Notably, 77.1% of Cohen syndrome patients present with neutropenia, though ethnicity influences prevalence 6. VPS13B mutations have also been identified in cases of retinitis pigmentosa, indicating additional ocular involvement beyond lens maintenance 7. Mitochondrial dysfunction and impaired lipid transfer appear central to disease pathogenesis.