Background: “Single” ventricle is one of the most difficult problems in contemporary pediatric cardiac surgery and cardiology. Usually that kind of patients need multistage operations. Material and methods: In the department of Congenital Heart Defects 53 patients at the age from 9 days till 30 years old with different complex congenital cardiac malformations were selected for univentricular repair. Left ventricular (LV) morphology was present in 33 (62.3%), right ventricular(RV) morphology – nm in 17 (32.1%), and mixed morphology – in 3 (5.7%). Results: Totally 80 stage operations were done (18 - bidirectional Glenn and 6 – completion of Fontan) with 2 perioperative deaths (2.5%) in patients with complete atrioventricular canal with unbalanced ventricles. In all cases Fontan operation was performed in extracardiac conduit modification. The evolution of the surgical management of univentricular heart is discussed along with the indications, selection criteria, and operative approaches for staged palliation. Herein, we discuss the shot-term and long-term results and complications of Fontan operation. Conclusion: Staged Fontan operation can be performed with low mortality rate and acceptable quality of life for the patients with univentricular physiology. Short-term and long-term results depend on underlying cardiac and pulmonary anatomy.