The paper presents the course of pregnancy delivery and early postpartum period in a 23-year-old woman with lysinuric protein intolerance (LPI). The pregnancy was uneventful and resulted in a caesarean birth to a healthy baby at 37 weeks gestation. Nevertheless, the course of pregnancy in women with LPI is associated with a significantly increased risk of serious complications, including acute hyperammonemia, preeclampsia and postpartum bleeding, as well as fetus intrauterine growth retardation. In many cases, intensive metabolic monitoring and a proper diet with protein limitation and appropriate amino acids supplementation may significantly reduce the risk for both the mother and the newborn.
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