Neuroblastoma (NB) is the most common extracranial solid tumor in children. Despite a variety of treatments, patients with advanced stage disease still have a poor prognosis. The molecular mechanisms underlying NB pathogenesis are not fully known. Increasing evidence shows that long noncoding RNA (lncRNA) is important in multiple ways in NB progression. LncRNA could act as competitive endogenous RNA, serve as a scaffold for proteins, or participate in histone modification, thus affecting proliferation, migration, invasion, and differentiation of NB. Numerous lncRNAs polymorphisms are significantly associated with NB susceptibility. Differently expressed lncRNAs can be used to construct risk scores to evaluate patient outcomes. In conclusion, these lncRNAs have the potential to be diagnostic markers as well as promising therapeutic targets in the future.