Spinal cord tumors are relatively uncommon in children. These tumors have been associated with increased intracranial pressure in both children and adults. An infant presented initially with hydrocephalus and subsequently developed symptoms consistent with spinal cord abnormalities. Various proposed etiologies for increased intracranial pressure in spinal cord tumors are presented. Spinal cord tumors should be considered in the presence of childhood hydrocephalus and increased intracranial pressure without a clearly defined etiology.