Abstract: Neuroblastoma(NB), one of the most common solid tumors in children, has widely varied clinical manifestations. High-risk NB progresses rapidly; even with intensive myeloablative chemotherapy, relapse is still common and almost all are fatal.To discover effective drugs and improve the prognosis of NB, the critical molecules in the pathogenesis of NB need to be examined. Anaplastic lymphoma kinase(ALK) is a member of the insulin receptor superfamily of receptor tyrosine kinases.ALK abnormalities exist in a variety of tumors, such as anaplastic large cell lymphoma, rhabdomyosarcoma, inflammatory myofibroblastic tumor, and NB. The abnormal forms of ALK include gene fusion, gene amplification, protein overexpression, and gene mutation.These abnormalities play important roles in the development of these tumors.Tyrosine kinase inhibitors as anti-cancer therapy for clinical applications and new specific small-molecule inhibitors of ALK have been developed.Consequently, the relationships of ALK aberrations with NB development and targeted ALK treatments for NB have received increased attention.This article mainly reviews the studies on the relationship between ALK abnormalities and NB development.