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This review provides a structured overview of major advances in human CDG reported from 2023 to 2026, including newly recognized CDG, emerging biochemical and pathophysiological insights, advances in diagnostic strategies and biomarkers, and progress in treatment. Recent developments include the recognition of RPN1\u2010CDG, UGGT1\u2010CDG, and DHRSX\u2010CDG, refinement of phenotypes in established disorders, including PMM2\u2010CDG, ALG13\u2010CDG, ALG8\u2010CDG, GMPPA\u2010CDG, TRAPPC11\u2010CDG, and PGAP3\u2010CDG, and increasing use of glycomics, glycoproteomics, cellular models, and multi\u2010omics approaches. Therapeutic progress remains uneven: no FDA\/EMA\u2010approved disease\u2010modifying therapy is currently available for any CDG, and most interventions remain off\u2010label, experimental, or supported by limited observational evidence. Despite substantial progress, most CDG still lack disease\u2010modifying treatment, validated biomarkers, and prospective natural history data.<\/jats:p>","DOI":"10.1002\/jimd.70256","type":"journal-article","created":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T08:32:30Z","timestamp":1790757150000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Congenital Disorders of Glycosylation (\n                    <scp>CDG<\/scp>\n                    ): State of the Art in 2026"],"prefix":"10.1002","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1849-8375","authenticated-orcid":false,"given":"Patryk","family":"Lipi\u0144ski","sequence":"first","affiliation":[{"name":"Third Department of Pediatrics Center of Postgraduate Medical Education  Dziekan\u00f3w Le\u015bny 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