Biallelic loss-of-function variants in the RINT1 gene have been implicated in acute liver failure in the pediatric population (1) and were recently described to lead to a complex form of HSP in 3 children with early-onset spastic paraplegia, ataxia, optic nerve hypoplasia with significant vision impairment, dysmorphic features, and a thin corpus callosum (2). The gene discussed is RINT1; the disease is hereditary spastic paraplegia.