Background:Congenital chylous ascites is a very rare condition that has been well recognized as early as the 1950s, and was mostly called “Congenital chyloperitoneum” during that time. Few cases have been reported during the 1960s, and in 1967, Craven et al attributed the development of congenital chylous ascites to obstruction of the cisterna chyli and chylous reflux into the peritoneal space and small intestine and they demonstrated lymphangiographically. Patients and methods: The use of a unique dietary therapy for the treatment of congenital chylous ascites is briefly described and the relevant literature is described. Results:An infant with congenital chylous ascites was seen at about the age of two and half months. He was initially treated with intravenous somatostatin infusion, and thereafter by repeated paracentesis, and continued to receive breast and bottle milk feedings. However, when he was first seen, ultrasound was still showing moderate ascites. The boy was successfully treated a combination of a unique form of very low-fat diet and octreotide acetate subcutaneous injections. Treatment was not associated with a negative effect on growth or development, and no evidence of nutritional deficiency was observed. Conclusion: The case number 112 of congenital chylous ascites is reported and its unique therapeutic approach is described.