Pulmonary embolism is an uncommon but potentially life-threatening event in children. There has been increasing awareness of pulmonary embolism in children with improved survival in children with systemic disease and advancements in diagnostic modalities. However, literature regarding pulmonary embolism in children is sparse, and thus current guidelines for management of pulmonary embolism in children are extrapolated from adult literature and remain controversial. This article reviews the background and pathophysiology of venous thromboembolism, as well as current diagnostic approach and recommended management of pulmonary embolism in children.