Objective To investigate the misdiagnosis of neuroblastoma (NB) in children and analyze the causes of misdiagnosis. Method: A retrospective analysis was conducted on the clinical data of 20 children with NB admitted to Shanxi Children's Hospital from January 2014 to December 2017, and relevant literature was reviewed. Among the 20 cases of NB, 7 were confirmed by postoperative pathological biopsy, and 13 were confirmed by B-mode ultrasound, CT, or bone marrow aspiration cytology. Among them, 12 were misdiagnosed in the early diagnosis. Conclusion: Early misdiagnosis is is more common in children with NB, and diagnosis requires a combination of multiple methods, resulting in poor prognosis.