Abstract: In recent years, the incidence of pediatric malignancies has gradually increased. High-risk patients have poor prognosis, and the main effect of treatments such as surgery and radio-chemotherapy has reached a plateau phase. Targeted therapy, with its reliable efficacy and mild toxicity, has become one of the most promising approaches. Anaplastic lymphoma kinase (ALK) is a receptor tyrosine kinase, and its abnormal forms include gene fusion, gene mutation, gene amplification, and protein overexpression. These aberrations play important roles in the development of childhood cancer. With the application of ALK inhibitors in clinical anti-tumor therapy, targeted ALK treatment has received increased attention. This article aims to review various studies on the relationship between ALK aberrations and pediatric malignancies.