Hemolytic anemia due to deficiency of erythrocytic pyruvate kinase is a rare autosomal recessive disorder. Pregnancy complicated by pyruvate kinase deficiency is rare; there are only 8 reported cases in the literature. A case is described that was characterized by increased hemolysis during pregnancy, requiring blood transfusions before and after delivery, and complicated by severe preeclampsia at term. Fetal and maternal outcome was successful. Increased hemolysis and favorable perinatal outcome occurred in all 8 reported cases.