A case is described of 68 years old woman suffering from a peculiar xanthogranulomatous process terminated with fatal renal complication. The clinical symptomatology was not characteristic and did not lead to diagnosis during the life. The process was localized around the cerebral basilar arteries, and was also in close connection with the adventitia of the thoracic aorta and of coronary arteries. Identical xanthogranulomatous infiltrate was found in the peripelvic fat of both kidneys where it led to stenosis of proximal parts of both ureters; less extensive infiltrates were present periportally in the liver and in the bone marrow. Histological findings are suggestive of the diagnosis of Erdheim-Chester disease. The differential diagnosis of the process is discussed.