Abstract Chylous ascites in infants and children has been the subject of 35 articles in the English language literature during the past 30 years. Analysis of the cases of 58 patients reveals that chylous ascites has been reported twice as frequently in infants as in older children, but that the clinical presentations, essentials of diagnosis, treatment alternatives, and mortality rates have been otherwise remarkably similar. Two thirds of patients treated without operation were cured, half of them within the first month of therapy. Even though the documented experience with total parenteral nutrition in infants and children with chylous ascites is remarkably scant, parenteral nutritional support appears to have decreased the hazard of medical therapy by maintaining an adequate nutritional intake while eliminating obligate losses consequent to repeated paracentesis. Patients in whom chylous ascites recurs, after resumption of a regular diet, should undergo surgical exploration, according to a defined strategy that includes visualization of the retroperitoneal origin of the superior mesenteric artery.