A two-year-old child presented with an acute inability to bear weight. Radiological investigation revealed a large cervicothoracolumbar syrinx of no known cause. During investigation, acute communicating hydrocephalus developed, which required a shunt. At surgery, a small thoracic spinal cord hemangioblastoma was discovered and excised. Complete recovery with collapse of the syrinx followed. The clinical features of this rare childhood tumor and its associated effects are discussed.