Cholestatic jaundice is a common presenting feature of neonatal hepatobiliary and metabolic dysfunction. Any infant who remains jaundiced beyond age 2 to 3 weeks should have the serum bilirubin level fractionated into a conjugated (direct) and unconjugated (indirect) portion in neonates mainly undirect bilirubin.

In cholestasis, the primary failure is of bilirubin excretion, resulting in excess conjugated bilirubin in the bloodstream and decreased bile salts in the gastrointestinal (GI) tract. Because of inadequate bile in the GI tract, there is malabsorption of fat and fat-soluble vitamins (A, D, E, and K), leading to vitamin deficiency, inadequate nutrition, and growth failure.

Our case is interesting present in first day of life with conjugate bilirubin which made us look for differential diagnosis, no family history of metabolic disease, 1st consequently, no dysmorphic feature, admitted in NICU as case of cholecystic jaundice for farther investigation, all investigations were done, and patient sent to higher center for farther management.