By performing whole-exome sequencing in a large patient cohort of a total of 2697 patients as well as using a Xenopus tropicalis model of congenital hydrocephalus, Singh et al. suggested that SMARCC1 is important for human congenital hydrocephalus pathogenesis, supported by a “neural stem cell” paradigm (Furey et al. 2018; Singh et al. 2024). Here, SMARCC1 is linked to congenital hydrocephalus.