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In this review, we offer a comprehensive overview of metabolic ataxias summarized by disease, highlighting novel clinical trials and emerging therapies with a particular emphasis on first-in-human gene therapies. We present disease-specific treatments if they exist and review the current evidence for symptomatic treatments of these highly heterogeneous diseases (where cerebellar ataxia is part of their phenotype) that aim to improve the disease burden and enhance quality of life. In general, a multimodal and holistic approach to the treatment of cerebellar ataxia, irrespective of etiology, is necessary to offer the best medical care. Physical therapy and speech and occupational therapy are obligatory. Genetic counseling is essential for making informed decisions about family planning.<\/jats:p>","DOI":"10.3390\/cells12182314","type":"journal-article","created":{"date-parts":[[2023,9,19]],"date-time":"2023-09-19T23:17:20Z","timestamp":1695165440000},"page":"2314","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Inborn Errors of Metabolism with Ataxia: Current and Future Treatment Options"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7397-045X","authenticated-orcid":false,"given":"Tatiana","family":"Bremova-Ertl","sequence":"first","affiliation":[{"name":"Department of Neurology, University Hospital Bern (Inselspital) and University of Bern, 3010 Bern, Switzerland"},{"name":"Center for Rare Diseases, University Hospital Bern (Inselspital) and University of Bern, 3010 Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Hofmann","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Bern (Inselspital) and University of Bern, 3010 Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janine","family":"Stucki","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Bern (Inselspital) and University of Bern, 3010 Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Vossenkaul","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology, Diabetes and Metabolism, Department of Paediatrics, Inselspital, Bern University Hospital, University of Bern, 3010 Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1358-1759","authenticated-orcid":false,"given":"Matthias","family":"Gautschi","sequence":"additional","affiliation":[{"name":"Division of Pediatric Endocrinology, Diabetes and Metabolism, Department of Paediatrics, Inselspital, Bern University Hospital, University of Bern, 3010 Bern, Switzerland"},{"name":"Institute of Clinical Chemistry, Inselspital, Bern University Hospital, University of Bern, 3010 Bern, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,9,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Hollak, C.E.M., and Lachmann, R. 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