INTRODUCTION: Epidemiologic studies have shown that patients with scleroderma have an increased risk of cancer. METHODS: The authors report eight cases and retrospectively review the published data on the possible association between breast cancer and scleroderma of the cutaneous and systemic forms. The records of all patients with scleroderma and breast cancer who were hospitalized during 1980-2002 in the Chaim Sheba Medical Center were surveyed. A literature search for breast cancer and scleroderma was performed. RESULTS: The clinical data of 65 women patients with breast cancer and scleroderma, eight of whom were hospitalized in the Chaim Sheba Medical Center and 57 who were reported in the literature, are summarized. In 29 patients (44%), scleroderma appeared before or simultaneously with the diagnosis of breast cancer. While a higher frequency of systemic scleroderma was described in this group, a higher rate of cutaneous scleroderma appeared in the group where scleroderma followed breast cancer diagnosis (p<0.001). CONCLUSIONS: Large-scale case-control studies are needed to substantiate the possible association between scleroderma-both cutaneous and systemic-and breast cancer.