Hereditary hemorrhagic telangiectasia ([HHT] or Osler-Weber-Rendu syndrome) can manifest as sudden onset of epistaxis or neurological deficit in a child with characteristic mucocutaneous telangiectasias or as an asymptomatic bruit with or without overlying cutaneous vascular lesions. The authors present a case study of a pediatric patient with HHT in whom a screening computerized tomography (CT) scan of the chest revealed an asymptomatic arteriovenous malformation (AVM) of the spine. An 18-month-old child with a strong family history of HHT, including fatal central nervous system (CNS) hemorrhage and pulmonary AVMs, presented with a cutaneous telangiectasia of the pinna. The child was subsequently screened for potentially morbid pulmonary and CNS AVMs by using chest CT scanning and brain magnetic resonance (MR) imaging. A spinal MR image revealed a perimedullary macro-AVF (MAVF) resulting in a large venous varix within the parenchyma of the thoracic spinal cord. A transarterial embolization of the fistula was performed using N-butyl cyanoacrylate and ethiodol. Postembolization angiography confirmed obliteration of the fistula, and MR imaging revealed thrombosis and reduction in size of the venous varix. There were no neurological sequelae due to the treatment. In families with HHT and a high risk of sudden severe morbidity or death from undisclosed pulmonary or CNS AVMs, screening chest CT scanning and CNS MR imaging should be considered. Interdisciplinary teams of neurosurgery and interventional radiology specialists should evaluate and treat such patients by using diagnostic and therapeutic angiography and, if necessary, surgery.