Long-term central venous access is an integral part of managing children with cancer, certain congenital malformations, and gastrointestinal malfunction, as well as for those who need long-term access to medications or blood products. Disease and patient-specific selection of access device type is important in minimizing complications and obtaining optimal outcomes. Because infection is the most common complication, enthusiasm has increased for developing methods to prevent infection, although without clear impact. Most infections can be treated successfully without device removal. Premature removal occurs more frequently with external catheters and may be minimized by techniques used for insertion and catheter care. Occlusion, if detected early, usually can be successfully managed by clot lysis.