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Summary Since the normal absorption of CSF occurs in the cerebral veins and venous sinuses, any obstruction to the normal flow and absorption of CSF will result in accumulation of CSF central to the site of obstruction. Such accumulation within the cranium is defined as hydrocephalus. A foal was presented with an enlarged and an abnormally-shaped skull, but with normal behavior. The filly's condition deteriorated. Radiographs showed a domeshaped cranial vault with compression of the frontal sinus region. Massive hydrocephalus with little normal cerebral tissue left was diagnosed with ultrasound. Surgery was attempted to relieve the pressure. Eventually the foal was euthanized. Post-mortem confirmed the radiographic and ultrasound diagnosis. Since there was a lack of demonstrable obstruction, the authors suspected the foal had suffered from the Arnold-Chiari syndrome.