1 INTRODUCTIONNeuroblastoma is an embryonic tumor derived from the neural crest with migratory neuroectodermal cells (1). It is one of the most common extracranial solid tumors in infants and children. More than 90% of neuroblastoma cases occur in children under 5 years old (2,3). The clinical signs and symptoms of neuroblastoma are highly dependent on the origin and spread of the tumor (4). Neuroblastoma with a primary lesion in the central nervous system is defined as primary central nervous system neuroblastoma (PCNSN), which is rare and carries a poor prognosis (5). In PCNSN, primary tumors located in the pineal gland are exceedingly rare, and there is no previous report thus far. Here, we report an adult patient diagnosed with primary pineal neuroblastoma who received surgical resection and postoperative radiotherapy.