Erdheim Chester disease (ECD) is an uncommon, non-Langerhans form of histiocytosis, characterized by long bone sclerosis. To date, only approximately some 100 cases have been reported. The diagnosis is based on its peculiar radiological and pathological features. ECD disease is rarely expected preoperatively. Although the ECD is an adult illness it can rarely be seen in the childhood period. We reported the case of a 10-year-old boy who presented headache, paraparesis and with diabetes inspidus for 6 years. As far as we know. the case presented here is the first published report of intracranial involvement and unilateral bone sclerosis with ECD in childhood. (c) 2006 Elsevier B.V. All rights reserved.