Sarcoidosis is a systemic disease of unknown etiology affecting all organ systems. Breast involvement accounts for less than 1 per cent of cases. In most cases systemic symptoms precede the finding of breast involvement. Involvement of the breast as the primary manifestation of this disease is a rare occurrence. A review of the literature shows a total of 29 cases published in the English language literature between 1921 and 1993. In these cases, less than a third had the breast as the primary organ involved. All cases of sarcoid breast disease presented as breast masses. Biopsy was done in most cases to rule out a malignant breast lesion. In four of the cases reviewed, sarcoid lesions and malignancy coexisted. Pathological features of sarcoidosis are typical for noncaseating lesion; however, differentiation from tuberculosis may be difficult. The PPD and Kviem test should be performed to help come to a diagnosis. Patients with primary breast involvement are highly likely to have subclinical pulmonary disease. Pulmonary function testing, serum lysozyme, and serum angiotensin-converting enzyme levels are helpful in assessing activity of the disease, and directing therapy. We present a case of sarcoidosis of the breast as the primary manifestation of sarcoidosis, and a review of the literature to illustrate these points.