Whether or not the association of systemic scleroderma and carcinoma is fortuitous is a much debated subject. Since 1886, approximately 320 cases of this association have been published. The most frequent malignancy involved is lung cancer (102 cases), followed by breast cancer (52 cases), malignant blood diseases (46 cases), cancer of the oesophagus (20 cases) and other gynaecological or gastrointestinal malignant tumours. A recent epidemiological study has shown that lung cancer is significantly more frequent than other malignancies, but statistical data on the latter are lacking. However, it would be wise to recommend that patients with systemic scleroderma should be regularly examined for gynaecological, haematological and gastrointestinal malignant diseases.