The most common cause of arterial thromboembolism in the pediatric population is secondary to arterial catheterization. Non-catheter-related arterial thromboembolism is rare. The frequency of this complication has paralleled the advances in pediatric tertiary care that has lead to increasing numbers of children surviving life-threatening primary illnesses. Previously, these events were very rare and children were managed according to adult protocols. It is now known that the pathophysiology of thrombosis in children differs from that of adults and indeed is different for that of various age groups within the pediatric population, therefore requiring a unique diagnostic and therapeutic approach. This review highlights the available published data to support an evidence-based approach to the complication of arterial thromboembolism in the pediatric population, and underscores the urgent need for further randomized controlled trials.