Neuroblastoma is an embryonic tumor of the peripheral sympathetic nervous system. It is the most common extracranial solid tumor of childhood and accounts for up to 15% of all pediatric cancer fatalities. The manifestation of neuroblastoma is variable depending on the location of the tumor and on the presence or absence of paraneoplastic syndromes. The prognosis of neuroblastoma is also highly variable, ranging from spontaneous regression to widespread metastatic disease that is unresponsive to treatment. The age of the patient, stage of disease, histopathologic results, and multiple biologic factors contribute to the presurgical and pretreatment risk stratification of a patient with neuroblastoma. Multimodality anatomic imaging with ultrasonography, computed tomography, and magnetic resonance imaging, as well as functional or metabolic nuclear imaging, are essential to determining the risk status of a patient with neuroblastoma. Patients at low risk of metastasis or death receive minimal intervention and those at high risk receive multimodality treatment. New immunotherapeutic techniques and nuclear medicine-targeted therapies have emerged and are demonstrating promising response rates for patients at high risk. This article reviews updates in the diagnosis, management, and treatment of neuroblastoma that have evolved over the past 2 decades, including emphasis on presurgical risk stratification, genetic evaluation of tumors, and the use of modern, high-quality, advanced imaging modalities. ©RSNA, 2018.