The results of surgical treatment of four children with chylous ascites unresponsive to medical management are presented. Peritoneovenous shunts were inserted in three patients, two of whom had the ascites resolved. The third patient was a 4-year-old girl with widespread mucin-secreting carcinomatosis, the first reported case in the literature combined with chylous ascites, whose shunt failed to relieve the ascites. A review of the literature comprising 92 cases of chylous ascites in children and a proposed scheme of management is presented.