Three cases of intraspinal tumors associated with hydrocephalus were encountered and treated. This review includes 67 cases, including these 3 cases, and discusses the pitfalls associated with intraspinal tumors presenting with hydrocephalus. These tumors are not easily diagnosed, especially in cases where the chief complaint, due to hydrocephalus, is headache or dementia, and does not include back pain. Among the 67 cases reviewed, symptoms suggesting normal pressure hydrocephalus were particularly noticed in patients over 40 years old. In cases presenting with headaches or dementia, back pain corresponding to the tumor location may appear after a shunt operation or external ventricular drainage. This postoperative back pain is important in indicating the presence of an intraspinal tumor.