PNETs of the spinal cord are aggressive and local recurrence and/or leptomeningeal spread is common. Primary spinal PNETs are extremely rare and most cases involving the spinal cord are drop metastases from primary intracranial tumors by cerebrospinal fluid. Herewith, we present a 40-day-old infant with multilevel primary spinal PNET at Th12-L1 and L5-S1 levels associated with hydrocephalus occurring nearly 15 days after the operation. According to our knowledge this is probably the first case harboring all these pathologies. Multilevel primary intraspinal PNET in an infant is even rarer and can be associated with hydrocephalus that occurs during the postoperative period.