The central venous catheter (CVC) has become an integral part of various long term parenteral therapies including chemotherapy and parenteral nutrition. In pediatric patients with a long term CVC, multiple repeated accesses and catheter-related complications may lead to difficulty in reestablishment of a line. Strategies in CVC management in these patients should begin with choosing an appropriate catheter according to its purpose, choosing the right access site and prompt treatment of potential complications, especially catheter-related thrombosis. In patients with severe restriction of the superior vena cava and its tributaries, end-stage central venous access is diagnosed. Management of this situation requires a multidisciplinary team and alternative routes of venous access including access through small collateral veins, or through an unusual vein such as the hepatic vein or a gonadal vein, and/or use of alternative surgical techniques. This article provides a comprehensive review regarding the current approach and surgical options in pediatric patients with end-stage central venous access.