Due to the rarity of congenital chylous ascites and the lack of standards in diagnosis and therapy, this disease constitutes a medical challenge and individual therapy seems to be extremely important. A late preterm newborn with antenatally diagnosed ascites was born and chylous ascites was diagnosed after feeds were started. The baby was managed initially with nil per oral, parenteral nutrition and octreotide, followed by adding MCT formula feeds. Considering the rarity of neonatal chylous ascites and the non-uniformity in management plans and follow up, more case reports need to be published. Also, MCT formula, the main stay of management has to be discontinued as soon as possible with gradual introduction into breast feeds or normal newborn formula milk as long chain fatty acids are essential for optimal brain growth in newborns.
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