Neurodegeneration in SCA14 is thought to result from defective aggregation and disrupted synaptic activity.6, 7 Clinically, SCA14 presents with slowly progressive, early‐onset, mainly pure cerebellar ataxia.8 Some complex ataxic phenotypes have been associated with PRKCG mutations, including focal, task‐induced dystonia, myoclonus, and pyramidal syndrome in single cases or small cohorts.1, 9. Here, PRKCG is linked to cerebellar ataxia.