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. Here, CLCN2 is linked to cerebellar ataxia.