OBJECTIVE: To investigate the significance of risk grouping and treatment by subgroup in neonatal tumors. METHODS: The clinical data of 37 children with neonatal adrenal neuroblastoma (NB) admitted between May 2003 and April 2015 were retrospectively analyzed and followed up. There were 22 males and 15 females; 23 cases on the right side of the lesion and 14 cases on the left side; ages 9-118 d (mean 31.2 d). According to the INSS staging system, 32 cases were stage I (including 2 cases of in situ NB) and 5 cases were stage IIa. Pathology was NB according to the Shimada classification, with 31 cases of well-organized (FH) and 6 cases of poorly organized (UH). N-myc gene amplification was negative in all cases. Age at diagnosis, imaging, tumor markers, intraoperative exploration of peritumor tissue infiltration, surgical management, peritumor lymph node metastasis, follow-up and tumor recurrence were analyzed. RESULTS: All children underwent complete stage I tumor resection + lymph node dissection for retroperitoneal enlargement. In one case, the tumor and the affected kidney were resected together because the tumor infiltrated the ipsilateral kidney, and no tumor recurrence was observed in the postoperative follow-up. One of them was found to have multiple skin nodules 11 months after surgery, and the tumor recurrence was confirmed by pathology after surgical resection, followed by 6 courses of chemotherapy. In the other case, a liver lesion was found 3 months after NB of the right adrenal gland, which was considered as tumor recurrence and chemotherapy was administered directly. All children were followed up for 1-51 months, with a mean of 29.3 months. All children survived, including 35 tumor-free cases and 2 cases with recurrence and tumor. Conclusion: 90% of neonatal NB is of adrenal origin, and most of the tumors found in early stages are relatively limited, mostly stage I tumors, with few bone metastases and very few stage 4 and 4s tumors. The majority of neonatal adrenal NB belongs to the low-risk group, which should be treated by simple surgical resection of the primary focus and necessary supportive therapy, and if necessary, the severely involved ipsilateral kidney can be removed to achieve complete resection of the tumor, and postoperative chemotherapy and radiotherapy can be dispensed with, but regular postoperative follow-up should be emphasized. This treatment plan not only avoids the high risk of postoperative chemotherapy and related complications in neonates, but also is a reasonable and effective treatment and cure. In children with postoperative recurrence, timely remedial chemotherapy and reoperation can still cure and control tumor progression as they have already passed the neonatal period.