Isolated ventricular non-compaction is a rare cardiomyopathy associated with left heart failure, severe arrhythmias and thromboembolism. We report about our interdisciplinary strategy in a patient with severe isolated ventricular non-compaction cardiomyopathy scheduled for caesarean section in general anaesthesia. Monitoring included placement of an arterial line, a central venous catheter and a pulmonary artery catheter with pacing option. Small introducer gates were placed in the femoral artery and vein to facilitate quick percutaneous institution of extracorporeal life support via extracorporeal membrane oxygenation in case of acute cardiac failure refractory to medical treatment. Inotropic pharmacological therapy with 3 µg/kg/min dobutamine and 0.25 mg/kg/min milrinone was started before surgery. Induction of general anesthesia and rapid sequence intubation was performed with an analgesic dose of 0.5 mg/kg S ketamine, 0.25 mg/kg etomidate and 5 mg rocoronium followed by 1.5 mg/kg succinylcholine. This regimen provided completely stable hemodynamics in this critical period until delivery of the child and continuation of anaesthesia with continuous infusion of propofol and remifentanyl. The current strategies, particularly the preparation for femoro-femoral extracorporeal membrane oxygenation, may be considered in similar cases with a high risk of acute cardiac decompensation which may be refractory to medical treatment. Anaesthesiologist involved in performing caesarean section in women with complex cardiac disease, should encompass extracorporeal membrane oxygenation standby in management of the perioperative period.