We present a case report of anomalous mitral arcade in a live born former recipient of twin–twin transfusion syndrome. At 33+0 week of gestation fetal ultrasound demonstrated that she had a large mitral insufficiency, decreased movement of the lateral cusp of the mitral valve and dilated left atrium. The twins were delivered by caesarean section at week 33+1 due to fetal distress. The former recipient twin developed decompensated heart failure during her first day of life and was transferred to a surgical paediatric heart centre. Her clinical condition rapidly deteriorated, and she died of congestive heart failure 3 days old. Prenatal signs of anomalous mitral arcade in a recipient of twin–twin transfusion syndrome should warrant preparation of a critically ill neonate, including parental counselling and in utero transfer to surgical paediatric heart centre. There is a surgical treatment option available for neonates, but the experience with this technique is still very limited with a high risk of morbidity and mortality.