BACKGROUND Pregnancy is contraindicated in uncorrected tricuspid atresia. Even following palliative surgery with the Fontan procedure, only four pregnancies have been reported. METHODS A 32-year-old female presented at 8 weeks' gestation in her first pregnancy with a history of the Fontan procedure performed seven years earlier for cardiac decompensation despite two previous palliative procedures for tricuspid atresia. She remained class I with no deterioration and required no medication throughout the pregnancy. Amniocentesis for positive maternal alpha-fetoprotein screening was normal. The pregnancy was complicated by antepartum hemorrhage at 29 weeks and premature rupture of membranes at 33 weeks, resulting in preterm labor and delivery of a 2.5-kg infant five days later. Apart from postpartum hemorrhage, the puerperium was uneventful, and the patient was well 20 weeks after delivery. CONCLUSIONS Five similar pregnancies, including this one, have been reported. Although all were successful, three infants were preterm and two growth retarded. This observation suggests that for these patients, pregnancy must be monitored closely even though the mothers may be hemodynamically stable.