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The prenatal sonographic diagnosis of Turner syndrome usually depends upon the discovery of a cystic hygroma or nonimmune hydrops fetalis. This report describes isolated fetal ascites as a newly recognized presentation of the disorder. Intrapartum fetal paracentesis permitted atraumatic vaginal birth. The etiology of the ascites in this case was congenital intestinal lymphangiectasia, consistent with the generalized lymphatic hypoplasia previously described in Turner syndrome.