Whole exome sequencing identified a pathogenic variant, p.Thr267Met, in <i>ITPR1</i> in both sisters, establishing their diagnosis as SCA29.<h4>Conclusions</h4>We report the identification of a family of Māori descent with a mutation causing SCA29, extending the worldwide scope of this disease. The gene discussed is ITPR1; the disease is spinocerebellar ataxia type 29.