We report a case with a giant spinal neurinoma located between the foramen magnum and fifth thoracic levels. The patient did not develop any neurologic symptoms until 16 years after the onset of hydrocephalus. The latter, successfully treated with a ventriculoperitoneal shunt, was associated with a cerebrospinal fluid protein of 400 mg/dL and was in all probability the first sign of the spinal cord tumor.