Pentalogy of Fallot is a cyanotic congenital heart disease that has guarded prognosis without surgical intervention in infancy. Women with uncorrected defects rarely survive into childbearing age and pregnancy in this group is associated with a high rate of perinatal loss. Physiological cardiovascular changes in pregnancy can lead to maternal haemodynamic instability with subsequent adverse cardiac sequelae with or without fetal decompensation. Optimum management and pregnancy outcomes in mother with uncorrected Pentalogy of Fallot and twin pregnancy have not been described in the literature. We describe a successful case of monochorionic diamniotic twin pregnancy in an affected woman who has not undergone surgical repair. Her pregnancy progressed without any adverse cardiopulmonary complications. Her caesarean delivery and postpartum recovery were favourable, with successful birth of two healthy babies at 35.7 weeks. This case emphasises the importance of a multidisciplinary team, especially of obstetricians with expertise in high-risk pregnancies, adult congenital heart disease cardiologists and anaesthesiologist.

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