The management of a heterogenous group of patients referred to as functional single ventricle varies with regard to morphological, clinical and haemodynamic findings. The application of appropriate palliative and staging operations, a better understanding of the Fontan physiology, and introduction of new techniques such as the fenestration procedure have resulted in marked improvement in the course and prognosis of the patients. In this review, current therapeutic options based on haemodynamic features and risk factors in patients with a functional signle ventircle are presented.