Science and Education Publishing American Journal of Medical Case Reports 2374-216X 3 5 2015 04 16 Late Onset Ornithine Transcarbamylase Deficiency Accompanying Severe HyperammonemiaAfter Cesarean Section: Case Report 133 136 EN Birsen Dogu Marash Life Hospital, Kahramanmaras, Turkey Nezir Y?lmaz Sabriye Ozcekic Hafize Oksuz AJMCR2015354 10.12691/ajmcr-3-5-4 2015 03 25 2015 04 04 2015 04 16 Hyperammonemia is one of the common complications of porto-systemic shunt or liver failure. In patients without liver failure or porto-systemic shunt, hyperammonemia can be caused by urea cycle disorders. Nitrogen excretion pathway or enzyme deficiency disorder can result in any hyperammonemia, coma, or death that may cause severe clinical encephalopathy presenting with neurological symptoms. The most common cause of genetic disorders in the urea cycle is ornithine carbamoyltransferase deficiency. In this article, 28-year-old female patient who had late-onset ornithine carbamoyltransferase deficiency with severe acute hyperammonemia resulting coma and death after cesarian section is described. In addition, treatment of hyperammonemia including sodium benzoate, arginine, and hemodialysis were discussed.