We here report on a neurodevelopmental disorder in two sisters with a homozygous nonsense variant c.376C > T; p.(Gln126*) in the alternatively spliced exon 4 of VLDLR. The phenotype in both siblings was less severe than previously reported for the VLDLR cerebellar hypoplasia [2]. The gene discussed is VLDLR; the disease is neurodevelopmental disorder.