Objective To analyze the clinical characteristics and prognosis factors of high-risk neuroblastoma (NB) in children. Methods A retrospective analysis of clinical data collected from children with high-risk NB from May 2017 to May 2022. Follow-up was conducted until February 1,2023. Prognosis-related factors were analyzed by comparing different clinical characteristics, treatment regimens, and survival status. Results Sixty high-risk NB children were included in this study, consisting of 36 males (60.0%) and 24 females (40.0%). The median age at diagnosis was 45 (8-103) months, median follow-up time was 39 (2-65) months, median time to disease progression/recurrence was 18 (2-62) months; and the median survival time was 41 months.. The 1-year, 2-year and 3-year overall survival (OS) rate were 76.4%, 65.6% and 54.8% respectively. The event-free survival (EFS) rate at 1 year, 2 years, and 3 years were 63.0%, 41.9% and 31.6% respectively. As of February 1, 2023, there were 34 survivors (56.7%) and 26 deaths (43.3%), with 17 of the deaths (65.3%) due to recurrence. Univariate survival analysis showed that pathological type, clinical stage,surgical status at initial diagnosis, bone marrow metastasis status, MYCN amplification, anemia status, NSE level, LDH level, and intracranial metastasis at relapse were adverse prognosis risk for high-risk NB (P<0.05). Cox multivariate analysis showed MYCN amplification, and bone marrow metastases at initial diagnosis were independent prognostic factors for high-risk NB (P=0.017). Conclusion The 3-year OS rate of high-risk NB children was 54.8%, High-risk neuroblastoma has a poor overall prognosis, with recurrence being the main cause of death. MYCN amplification is an independent adverse prognostic factor for high-risk NB.