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Late\u2010onset patients have a better prognosis but develop common long\u2010term complications, including neurological deterioration, chronic kidney disease, pancreatitis, optic neuropathy, and chronic liver disease. Of note, oncogenesis has been reported anecdotally in organic acidurias. Here, we present three novel and two previously published cases of MMA patients who developed malignant liver neoplasms. All five patients were affected by a severe, early\u2010onset form of isolated MMA (4 <jats:italic>mut<\/jats:italic><jats:sup>0<\/jats:sup>, 1 <jats:italic>cblB<\/jats:italic> subtype). Different types of liver neoplasms, that is, hepatoblastoma and hepatocellular carcinoma, were diagnosed at ages ranging from infancy to adulthood. We discuss pathophysiological hypotheses involved in MMA\u2010related oncogenesis such as mitochondrial dysfunction, impairment of tricarboxylic acid cycle, oxidative stress, and effects of oncometabolites. Based on the intriguing occurrence of liver abnormalities, including neoplasms, we recommend close biochemical and imaging monitoring of liver disease in routine follow\u2010up of MMA patients.<\/jats:p>","DOI":"10.1002\/jimd.12143","type":"journal-article","created":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T15:55:02Z","timestamp":1561996502000},"page":"793-802","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Liver neoplasms in methylmalonic aciduria: An emerging complication"],"prefix":"10.1002","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1877-2976","authenticated-orcid":false,"given":"Patrick","family":"Forny","sequence":"first","affiliation":[{"name":"Metabolic Medicine Department Great Ormond Street Hospital, Institute of Child Health University College London  London 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Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Baruteau","sequence":"additional","affiliation":[{"name":"Metabolic Medicine Department Great Ormond Street Hospital, Institute of Child Health University College London  London UK"},{"name":"National Institute of Health Research Great Ormond Street Hospital Biomedical Research Centre  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Grunewald","sequence":"additional","affiliation":[{"name":"Metabolic Medicine Department Great Ormond Street Hospital, Institute of Child Health University College London  London 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