Background The association between spinal cord tumors and hydrocephalus is a rarely reported phenomenon. Diagnosis in this group of patients is difficult as they present with findings of an intracranial pathology and the symptoms of a spinal lesion may be absent. Case Report We report two cases of spinal cord tumors presenting with visual disturbance and findings of increased intracranial pressure. Discussions Mechanisms describing the relationship between spinal cord tumors and increased intracranial pressure have been explained. Most of the literature reported marked regression of these manifestations after tumor excision. Conclusions Spinal cord tumors associated with hydrocephalus and papilledema are rare conditions. The diagnosis of these conditions may be difficult or confusing because the symptoms referable to the spinal lesion may be minimal. Meticulous history taking, examination, and investigations are mandatory to diagnose this entity.