The association of malignancy with collagen diseases has been a focus of interest. Especially, relation between dermatomyositis and malignancy is well-recognized. I reviewed here about the coexistence of SSc and malignancy in literature. I would like to raise four points as characteristics of the relationship. (1) Special attention should be paid to the patients who is late-onset, with advanced skin sclerosis (ex. Barnett III type) and male. (2) There are three generalized mechanisms of the association. The first is the predisposition of the malignancy on the basis of organ fibrosis, such as pulmonary fibrosis and lung cancer. The second is the nature of paraneoplastic syndrome. Lastly, immunological derangement inherent to SSc might cause carcinogenesis. (3) It has been documented that the interval of the onsets of SSc and breast cancer is extremely short. (4) Longstanding reflux esophagitis and Barrett's esophagus with SSc is suspected to predispose to esophageal carcinoma.