Abstract Intramedullary glial neoplasms affecting the entire cord from the cervicomedullary junction to the conus are termed “holocord tumors” and those diagnosed as pilocytic astrocytoma are rare. Herein, we present a 13-year-old girl with a tumor extending from the cervicomedullary junction to the conus which was partially resected in a four-stage approach. Histopathological examination of all specimens resulted in diagnosis of a pilocytic astrocytoma. Although no signs of atypia were present, an elevated proliferative activity of endothelial vessels was noted. Residual parts of the tumor showed progress making additional surgery necessary. Therapy and its consequences are discussed and an overview of the literature of these rare longitudinally extensive intramedullary lesions is given.