The association of cancer and connective tissue disease is well known, the most frequent being certainly with dermatomyositis. The association cancer and PSS is more controversial. The incidence of neoplasia in that group seems to be comparable with the general population but the proportion of certain types of cancer is different, and the temporal relationship with the apparition of symptoms of PSS is stunning. The hypothesis actually in favor is an imbalance of the immune system, which cause the diminution of the immune surveillance and the apparition of cancer and a concomitant dysregulation of the system, causing the fibrosis of the PSS. We describe a 75 year-old white female who developed a colic adenocarcinoma; she also had, concomitantly, a systemic scleroderma, with sclerodactyly and pulmonary fibrosis. The patient corresponded to the criteria of the American Rheumatism Association for progressive systemic scleroderma (PSS). The prognosis of patients with PSS depends on their systemic involvement but also, we believe, in the more aged group, on the apparition of a neoplasia.