Thromboembolism is an established complication of long-term parenteral nutrition (PN) administration which requires central venous lines in the pediatric population. Predisposing factors that increase the risk of thrombosis, as well as prophylaxis and treatment guidelines in this specific population, are not clearly defined. We performed a computerized search of PubMed, OVID databases, and pertinent articles from reference lists of related review papers. This review summarizes currently available data on the rates of thromboembolism in the pediatric population receiving long-term PN and concludes that control of factors such as location of catheter, duration of nutrition support, and prophylaxis with heparin or anticoagulants may reduce the rates of thrombosis in this patient population, although most data on the matter are inconclusive.