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Summary Erdheim-Chester disease (ECD) belongs to the malignant polyostotic sclerotic forms of non-Langerhans histiocytosis. During abnormal prolipheration of pathologic histiocytes (foam cells), involved structures become hypertrophic with increased density followed by scarring. Mostly the diaphysis and metaphysis of long bones of lower limbs with typical pain are involved. According to the WHO, the disease is classified as histiocytic neoplasia. We refer on a 74-year-old female patient with expansive process in the region of upper clivus and sphenoidal bone on the right side. Histological testing confirmed this very rare disease that affects about 600 patients over the world. Key words Erdheim-Chester – foamy histiocytes – sclerosis – fibrosis