Xanthoma disseminatum is a non-familial disorder of non-Langerhans cell origin or a class II histiocytosis with unknown etiology, with just over 100 cases reported in the literature. Because of the rarity of this disease, there is no established treatment. We studied clinical manifestations and different treatments of xanthoma disseminatum from a series of cases, including our own patient. We studied 15 articles on treatment of xanthoma disseminatum. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results. Systemic medication with peroxisome proliferator-activated gamma receptors, statins, fenofibrate, chlorodeoxyadenosine, cyclophosphamide, doxycycline, and cyclosporine have also been reported, but none have proven particularly successful. Xanthoma disseminatum is usually benign and is often self-limiting. If the lesions are accessible to surgery, that is likely to give the best results. However, if the lesions are not accessible for surgical removal then carbon dioxide laser treatment may be considered. The choice of oral treatment should be made on the basis of the patient's condition, since none of them have proven particularly effective. Expectant management is justifiable as long as the lesions are limited to the skin.