The review of data available in the literature has shown that there has been a gradual increase in the number of reported cases of intramedullary cavernous angioma (ImCA) since the advent of Magnetic Resonance Imagining (MRI). However, a comparison between the number of studies on adult and pediatric ImCA cases reveals that the total number of pediatric ImCA cases reported to date is only 18 - quite smaller than the former. The purpose of this study is to present an outline of ImCA in pediatric age group; its clinical presentation, course, management and outcome and to report the 19th pediatric case with an ImCA. Another important point about the present case is the three intracerebral lesions she had and the familial pattern existed. A complete removal of the lesion was accomplished as demonstrated by postoperative MRI studies. At the follow-up examination, a spine and cerebral MRI was completed and it was clear that no postoperative complication occurred after surgery.