Rationale:Neuroblastoma is the most common extracranial malignant solid tumor that occurs during childhood. It arises from primitive cells and is found in the adrenal medulla and sympathetic ganglia of the sympathetic nervous system. Huge neuroblastoma in the retroperitoneum, especially adult involvement is extremely rare.Patient concerns:A 20-year-old female patient with complaints of left abdominal discomfort for 1 week was reported.Diagnosis:Multi-detector computed tomography (MD-CT) of the abdomen revealed a huge enhanced mass in the retroperitoneum. Histopathological findings showed neuroblastoma and immunohistochemical results were as follows: actin(–), CD34(–), CD99(–), CK(–), CgA(+), desmin(–), EMA(–), Ki-67(+, approximately 1%), NSE(+), S-100(+), Syn(+), and vimentin(–).Interventions:We performed a total surgical resection. The CYVADIC (cyclophosphamide, vincristine, adriamycin, and dimethyl triazeno imidazole carboxamide) and James (cyclophosphamide and vincristine) regimens had been administered to this patient.Outcomes:Postoperatively, the patient's symptoms were partially relieved and the patient experienced recurrence after 3 months. The patient did not respond to treatment and died 6 months after the operation.Lessons:Besides surgical resection, the treatment also included chemotherapy and radiotherapy. However, the optimal treatment remains controversial. Therefore, we should exert all our energies on the exploration of etiology and targeted drugs for this disease.