An estimated 17.6 per 100,000 new cases of childhood cancer are diagnosed each year in the United States. The major subtypes of childhood cancer include leukemia, central nervous system tumors, lymphoma, neuroblastoma, rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, Wilms tumor, Germ cell tumors, and other rare tumors. Despite improvements in the diagnosis and treatment of these tumors over the last 30 years, subsets of children still have poor outcomes and many others have significant morbidity. Dysregulation of apoptotic pathways has been shown to contribute to tumor formation as well as resistance to therapy in both pediatric and adult malignancies. Survivin, the smallest member of the inhibitor of apoptosis protein (IAP) family, is highly expressed in diverse cancers and correlates with decreased patient survival. Here, we review the current literature on Survivin expression in pediatric cancer, its relationship to clinical outcome and potential therapeutic options to target this protein in pediatric cancer.