Pulmonary embolism (PE) in children is a rare condition with potential for high mortality. PE incidence is increasing owing to increased survival of children with predisposing conditions, increased use of central venous catheters, and improved awareness and recognition. Although pediatric PE is distinct from adult PE, management guidelines in children are extrapolated from the adult data. Treatment includes thrombolysis or thrombectomy, and pharmacologic anticoagulation. Ongoing clinical trials are evaluating the use of direct oral anticoagulants in children. Further research is required to develop pediatric-specific evidence-based guidelines for diagnosis and management of PE.