Abstract: Neuroblastoma is the most common extracranial solid tumor in childhood originating from the neural crest.Neuroblastomas are heterogeneous in terms of location, histopathologic appearance, biological characteristics, and clinical presentations.For patients in the low-risk group, the 5-year survival rate is higher than 95%;however, the 5-year survival rate is approximately 40%-50% for patients in the high-risk group.Patients with refractory or relapsed high-risk neuroblastoma continue to experience poor outcomes. Based on advancements in understanding the pathological and molecular characteristics of neuroblastoma, many new targeted therapies and prognostic biomarkers have been applied in neuroblastoma treatment.This review focused on recent advances in the molecular biology of neuroblastoma and targeted therapies that have emerged in clinical trials.