A 16-month-old girl with cutaneomeningospinal angiomatosis became paraplegic because of the intraspinal arteriovenous malformation. Since skin hemangiomas occurred in three successive generations of the girl's family, this may represent the first inherited case of this condition. In children with signs of a spinal space-occupying lesion, the presence of a skin hemangioma, especially if it is in a corresponding dermatome, could be the clue to early diagnosis of a spinal angioma.