The lack of this variant in public databases, the physiochemical nature of the amino acid exchange and the partly pathogenic disease causing in silico predictions, together with the fact that SCA13 mutations are primarily found in the heterozygous state, indicate that the KCNC3 variant is the most likely disease-associated variant in our patient with an atypical cerebellar ataxia. Here, KCNC3 is linked to cerebellar ataxia.