Background: The incidence of relapsed neuroblastoma (NB) in the central nervous system (CNS) is less than 20%. Here, we presented eleven cases with CNS relapsed NB to better understand the CNS relapsed NB. Methods: Medical records from NB patients between January 1st, 2010 and December 31st, 2017 were reviewed to examine those diagnosed with CNS relapsed NB. The following patient information was extracted, including age, gender, histological subtyping, tumor stage, risk group, biomarkers, chemotherapy regimen and dosage, surgery details, treatment outcomes and follow-up, clinical manifestations of CNS relapsed NB, treatment protocols for CNS relapsed NB, and patient outcomes (including the cause of death). Results: From January 1st, 2010 to December 31st, 2017, eleven NB patients were diagnosed with CNS relapse at our department. All the eleven patients were classified to have high risk disease. The median time from the completion of chemotherapy to CNS relapse was 5 months. Five patients did not receive any treatment for the relapsed disease and finally died of it. Curative treatments were tried in six patients. At last, two patients with isolated CNS relapsed NB survived. Conclusions: The median time from the completion of chemotherapy to CNS relapse is very short in the present retrospective study. It seems that patients with isolated CNS relapsed disease have better outcomes. We suggest the temozolomide-topotecan combination as an add-one backbone for further salvage treatment in children with CNS relapsed NB, even though this combination is not superior to others in terms of its efficacy.