Xanthoma disseminatum is a normolipemic xanthomatosis classified in the group of non-Langerhans cell histiocytic disorders. Classic clinical findings are cutaneous and mucosal xanthomas and diabetes insipidus. Ocular, liver, bone marrow, respiratory, skeletal, and central nervous system involvement may accompany the classic findings of the disease. Skeletal involvement is a rare manifestation of the disease in children. We report on a 5-year-old boy with cranial bone involvement, proptosis and xanthomatous cutaneous lesions, treated successfully with systemic corticosteroids.