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Intramedullary spinal hemangioblastomas are often associated with syringomyelia. However, holocord syrinx associated with such lesions is a rare entity. We present two cases of dorsal spinal hemangioblastoma associated with holocord syrinx. Both the cases were treated with surgical excision which resulted in the resolution of holocord syrinx and good neurological recovery. In this case report, we discuss the various aspects of the diagnosis and surgical management in such cases. We also present an extensive review of literature of such lesions.