Background Women with an uncorrected single ventricle heart are at increased risk of adverse maternal and perinatal outcomes. Methods We report our experience of managing pregnant women with uncorrected single ventricles, during the time period 2011 to 2017, in a low-resource setting and compare pregnancy outcome with healthy concurrent controls. Outcomes assessed include the mode of delivery, maternal complications, neonatal death and birth weight. Results There were six pregnant women with uncorrected single ventricles who had a total of 14 pregnancies. There was one maternal death in a woman with atrioventricular-septal defect and Eisenmenger syndrome. Caesarean section rates and preterm delivery were similar, whereas perinatal loss and low-birth weight rates were higher among women with a single ventricle compared to healthy controls. Conclusion Unplanned pregnancies without prenatal counselling/care pose a challenge to physicians especially in low to middle income countries and with the high risk of morbidity/mortality, pregnancy should be discouraged.