The univentricular heart includes a spectrum of complex cardiac defects that are managed by staged palliative surgical procedures, ultimately resulting in a Fontan procedure. Since 1971, when it was first developed, the procedure has undergone several variations. These patients require lifelong management, including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function, together with multi-organ evaluation. As these patients enter middle age, there is increasing awareness of long-term complications and mortality. This review highlights the concept behind the staged surgical palliations, the unique single ventricle physiology and the long-term complications in this complex cohort of patients.