Tetralogy of Fallot (TOF) is the most common congenital heart defect associated with systemic cyanosis. During pregnancy and delivery, dramatic alterations in cardiovascular physiology occurred. Pregnancy in women with unrepaired TOF may have a worsening in right to left shunt with an increase of the cyanosis and may imply an elevated risk of maternal and fetal morbidity and even mortality. We report and discuss a case of a 26 years old woman with uncorrected TOF. A multidisciplinary team was involved in the management of the case with the aim to minimize maternal and fetal complications. One of the main endpoint was to maintain an adequate oxygen saturation and good haemoglobin levels. A caesarean section was performed at 30 weeks and 5 days of gestation without any maternal or fetal complications. During the puerperium, the woman had an embolic occlusion of the right middle cerebral artery with any sequelae.