A rare case of large bilateral sclerosing lipogranuloma with multiple calcifications of gluteal region is described in an old female patient affected by a cerebrovascular disease. The lesions appeared as firm, nontender, plaques, 9-10 cm in diameter, covered with hyperpigmented skin. This uncommon disorder is discussed on the basis of data obtained from an extensive literature review. The term "sclerosing lipogranuloma" was coined in 1950, and it defines a disease of the subcutaneous fat, which for a trauma or unknown reasons undergoes necrosis of fat cells with the release of fat droplets into intercellular spaces and a peculiar local sclerosing granulomatous reaction of fatty tissue. The cytosteatonecrosis and sclerosing lipogranuloma, post-traumatic or secondary to injection of exogenous oily substances, usually localized in the breast of women and in genitalia of men, are relatively well known. Sclerosing lipogranulomatosis of the orbita and eyelides, an infrequent but severe complication after endonasal surgery, has also been reported. Rarely, the lipogranuloma can be spontaneous or idiopathic or primitive. A particular form of genetic diffuse lipogranulomatosis is the Farber's syndrome, firstly described on 1947. In our patient, the absence of trauma seems to indicate a primitive lipogranuloma. The presence of an acute rheumatic syndrome responsive to corticosteroids, and the positivity of antimitochondrial autoantibodies are in accordance with the report of sclerosing multiple lipogranulomatosis associated with a lupus-like syndrome. Because of the long duration and the absence of acute local symptoms, this syndrome can be considered benign with favorable prognosis, but the physician should know it.