Introduction Wilson disease is a very rare inherited autosomal recessive disorder, with an incidence of 1 in 30,000 live births, associated with impaired copper metabolism leading to decreased biliary excretion and accumulation of copper in the liver and brain. Patients may be asymptomatic or might present with fulminant liver disease or neuropsychiatric illness. Untreated Wilson disease is related to infertility/subfertility/recurrent pregnancy loss. Aim To study the maternal and perinatal outcomes of Wilson disease in pregnancy. Materials and methods It is a retrospective observational study carried out in the Department of Obstetrics and Gynecology, St. John's Medical College Hospital, Bengaluru, India, between November 2010 and November 2015. Five patients with Wilson disease were identified during the study period. Pregnancy outcome was good in all these five women who were on regular treatment. Conclusion Patients with Wilson disease who receive regular treatment and who remain asymptomatic and conceive normally have favorable pregnancy outcomes. They merit regular surveillance and active management in higher centers with multidisciplinary approach involving gastroenterologists, obstetricians, neurologists, and intensivists. How to cite this article Vishnupriya KMN, Sheela CNR, Thayumanasundaram M. Maternal and Perinatal Outcome of Wilson Disease in Pregnancy: A 5-year Experience at a Tertiary Care Center. J South Asian Feder Obst Gynae 2017;9(4):318-322.