Here we describe a patient with optic atrophy, cataracts, ataxia, and peripheral and autonomic neuropathy associated with an autosomal dominant c.235C > G p.(Leu79Val) mutation in exon 2 of the OPA3 gene (National Center for Biotechnology Information [NCBI] reference sequence NM_025136.3). Here, OPA3 is linked to cerebellar ataxia.