A short summary of normolipemic xanthomatoses is exposed. A case of xanthoma disseminatum is reported. The typical clinical picture is described and numerous investigations are recorded. No association with any systemic disorder neither diabetes insipidus nor rheumatoid arthritis could be found. Histologically the granulomas consist of many multinuclear giant cells embedded in an infiltration of histiocytic cells and lymphocytes. Fat staining demonstrates lipid accumulation as a secondary phenomenon. Systemic corticosteroid therapy was effective in preventing the regrowth of the granulomas after their surgical removal.