Biallelic pathogenic variants in CLCN2 are associated with a rare autosomal recessive leucoencephalopathy with prominent ataxia1 recently named leucoencephalopathy with ataxia (LKPAT; Online Mendelian Inheritance in Man #615651). This evidence concerns the gene CLCN2 and leukoencephalopathy with mild cerebellar ataxia and white matter edema.