BackgroundIndividuals with variants of cytochrome c oxidase assembly factor 7 (COA7), a mitochondrial functional-related gene, exhibit symptoms of spinocerebellar ataxia with axonal neuropathy before the age of 20. However,COA7variants with parkinsonism or adult-onset type cases have not been described.Case presentationWe report the case of a patient who developed cerebellar symptoms and slowly progressive sensory and motor neuropathy in the extremities, similar to Charcot-Marie-Tooth disease, at age 30, followed by parkinsonism at age 58. Exome analysis revealedCOA7missense mutation in homozygotes (NM_023077.2:c.17A > G, NP_075565.2: p.Asp6Gly). Dopamine transporter single-photon emission computed tomography using a123I-Ioflupane revealed clear hypo-accumulation in the bilateral striatum. However,123I-metaiodobenzylguanidine myocardial scintigraphy showed normal sympathetic nerve function. Levodopa administration improved parkinsonism in this patient.ConclusionsCOA7gene variants may have caused parkinsonism in this case because mitochondrial function-related genes, such asparkinandPINK1,are known causative genes in some familial Parkinson’s diseases.