Juvenile xanthogranuloma is a histiocytic proliferative disease that predominantly affects the skin. Extracutaneous involvement is rare. We present the case of a 6-month-old infant with acute paraplegia. Magnetic resonance imaging showed an intraspinal extradural mass at midthoracic level with marked compression of the spinal cord. Complete tumor removal was achieved by emergency surgery and was followed by complete neurologic recovery. Histologic examination led to the diagnosis of a juvenile xanthogranuloma. To the best of our knowledge, an isolated intraspinal juvenile xanthogranuloma in the first 12 months of life has not been described before.