Congenital chylous ascites is a rare disease that results from abnormal development of the intra-abdominal lymphatic system. No gold standard treatment has been described so far, however, a combination of medium–chain triglyceride based diet or total parenteral nutrition along with octreotide and abdominal paracentesis is considered as a conservative management. This treatment is often a challenge to physicians since chylous ascites is often refractory and result in malnutrition and immune deficiency because of the loss of proteins and lymphocytes. We report a four-month old boy with congenital chylous ascites who was refractory to medical treatment with prolonged bowel rest, total parenteral nutrition, octreotide and repeated paracentesis. The baby well responded to surgical treatment with application of fibrin glue on the surface area of the leak site and was discharged after 2 month of hospitalization. When following up the patient had no recurrence of the ascites and he was growing up normally.