Leukoencephalopathy with ataxia (LKPAT), also known as <i>CLCN2</i>-related leukoencephalopathy, is a rare autosomal recessive disorder caused by pathogenic variants in <i>CLCN2,</i> which encodes ClC-2, a ubiquitously expressed chloride channel protein. This evidence concerns the gene CLCN2 and leukoencephalopathy with mild cerebellar ataxia and white matter edema.