OBJECTIVES To summarize a) epidemiology of arterial and venous thromboembolism, pulmonary embolism, and deep venous thrombosis in children; b) the risk factors for thrombosis in the pediatric intensive care unit; c) diagnostic techniques for arterial/venous thromboembolism; and d) the current recommendations for management and prevention of thromboembolic disease in critically ill children. DATA SOURCE Literature review, using National Library of Medicine PubMed and the following terms: arterial, venous thromboembolism; deep venous thrombosis; pulmonary embolism; thrombosis; as well as citations of interest from these articles. STUDY SELECTION Both pediatric and adult literature addressing thrombotic disease were reviewed. DATA EXTRACTION AND SYNTHESIS Articles were chosen for more extensive discussion when containing prospective studies, guidelines for practice, or data in critically ill patients. When data in children were unavailable, applicable data in adults were referenced. Due to the paucity of data in critically ill children, available adult and pediatric data were combined with institutional experience to provide suggestions for current practice and future inquiry. CONCLUSIONS Increasing awareness regarding the recognition and current approaches to management and prevention of thromboembolic disease in children is needed among pediatric intensivists, so outcome of these life-threatening processes might be improved.