Background: Crigler–najjar syndrome is a rare inherited disorder of congenital unconjugated hyperbilirubinemia and few cases of CNS with pregnancy have been reported till date.

Objective: To  report a case of maternal Crigler–Najjar syndrome in pregnancy, To review all the cases of CNS 1 and 2 reported in literature till date and evaluate maternal and fetal outcome in these cases.

Materials and Methods: PubMed and Google Scholar database was searched for case reports of CNS in pregnancy using  key words: Crigler–Najjar syndrome and/in pregnancy ,phototherapy, phenobarbital and pregnancy, till 12th November 2017.

Results: A total of 21 pregnancies were reported in 15 patients of CNS (excluding our patient). Maternal– fetal outcome was favorable in most cases with appropriate treatment with phototherapy and low dose phenobarbital.

Conclusion: CNS is a rare cause of jaundice in pregnancy. Serum bilirubin levels should be kept below 10mg/dl by judicious treatment for optimal outcome.