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We have collected all the cases present in the literature on the prenatal diagnosis of KTW, emphasizing common ultrasound findings that can guide the clinician and genetics to the prenatal counseling. Thus, we collected all the information about the postnatal prognosis and the necessity for treatment. Our review of 44 cases highlights the typical common features: hemihypertrophy, predominantly affecting the right leg, with cystic lesions extending to the trunk or upper limbs and rare internal organ involvement. Prenatal complications, including hydrops and polyhydramnios, emphasize the need for a careful ultrasound follow\u2010up. Despite no identified genetic mutation, genetic counseling and invasive testing are recommended. Mortality rate due to a severe complication known as Kasabach\u2010Merritt syndrome, underlines the importance of early diagnosis and accurate management strategies. Prenatal diagnosis of KTW, guided by ultrasound findings and genetic counseling, could help with informed decision\u2010making and optimal care planning.<\/jats:p>","DOI":"10.1002\/jcu.23864","type":"journal-article","created":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T20:43:14Z","timestamp":1730148194000},"page":"535-546","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Klippel\u2010Tr\u00e9naunay\u2010Weber Syndrome: Prenatal Diagnosis and Review of the Literature"],"prefix":"10.1002","volume":"53","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9177-8552","authenticated-orcid":false,"given":"Giuliana","family":"Orlandi","sequence":"first","affiliation":[{"name":"Gynecology and Obstetrics Unit, Department of Neuroscience, Reproductive Sciences and Dentistry, School of Medicine University of Naples Federico II  Naples 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