The presence of pulmonary arterial hypertension (PAH) in pregnancy is rare and signifies a high-risk pregnancy. Although the majority of mothers have knowledge of their condition before pregnancy, approximately one-third of patients are diagnosed during pregnancy. Termination of pregnancy should be discussed, and is often advised; however, a significant proportion of patients will choose to proceed with the pregnancy despite increased maternal and fetal mortality. Currently, most pregnant patients receive advanced therapy for treatment of PAH, particularly prostacyclin analogues. Particular attention is paid to volume status and blood loss and there has been a major trend toward delivery by cesarean section under controlled conditions involving an expert multidisciplinary team. The time of greatest maternal risk is in the first month after delivery. Transplantation of these patients in the nonpregnant state may be considered when those with idiopathic pulmonary hypertension have poor functional status despite optimal therapy and their projected 2-year survival is less than 50%. For patients with Eisenmenger syndrome, severe symptoms and an unacceptable quality of life may lead to transplantation.