A case of a nearly 3-year old girl with chylous ascites in neonatal period is reported. Repeated paracentesis were performed and the child was treated with a low fat diet. After time of 8 weeks ascites did not recur. It is purposed, that there was an abdominal lymphdysplasia. The 51 cases in the reviewed literature are analyzed in terms of symptoms, treatment, etiology and prognosis. It is suggested, that congenital chylous ascites should be treated conservative at first.