Heterozygous missense or frameshift mutations [3, 6] as well aspartial deletion [7] of AFG3L2 havebeen associated to spinocerebellar ataxia type 28 (SCA28), characterized by autosomaldominant inheritance [8] (https://www.omim.org).SCA28 [9] is characterized by young-adult onset, withcerebellar atrophy but no signs of cognitive impairment and sensory involvement [10]. Here, AFG3L2 is linked to spinocerebellar ataxia type 28.