We report here central nervous system (CNS) recurrence in neuroblastoma (NBL) after CD34(+) peripheral blood stem cell transplantation (PBSCT). Fifteen stage 4 NBL patients underwent CD34(+) transplantation with myeloablative chemotherapy consisting of carboplatin, etoposide, and melphalan. There were three primary site recurrences and five distant metastases including four brain metastases (two isolated CNS recurrences) at 4-7 months after CD34(+) transplantation. Three of four patients died of CNS progressive disease at 2, 8, and 9 months after recurrence and the remaining single patient was lost to follow-up. CNS recurrence in NBL is fatal and requires identification of risk factors and more effective treatment strategies.