A number of rheumatic disorders may appear as paraneoplastic syndromes, the most common being dermatomyositis or polymyositis. Systemic sclerosis is associated with a slightly increased risk of cancer, although its direct association with malignancies is controversial. We describe a case of a 57-year-old male with rectal adenocarcinoma and systemic sclerosis. Close temporal relationship between the initial presentation and parallel course of both conditions, as well as atypically rapid progression of systemic sclerosis symptoms, were observed in the reported case. The strict relation between these two conditions suggested that systemic sclerosis was a paraneoplastic syndrome rather than a concomitant morbidity in the presented patient. Current literature on systemic sclerosis coexisting with colorectal tumours is very limited, especially in the paraneoplastic setting.