Objective. To evaluate the incidence, the subspecificities and the clinical correlation of antineutrophil cytoplasmic antibodies in scleroderma patients. Methods. Sixty-two patients affected by scleroderma in limited or diffuse pattern have been screened for the presence of anti-proteinase 3 and anti-myeloperoxidase antibodies by ELISA method. Results. Two patients affected by diffuse systemic sclerosis were found to be antineutrophil cytoplasmic antibody-positive. One patient presented a weak positivity for anti-myeloperoxidase antibodies in the absence of renal impairment. One patient which clinical course is characterised by rapidly progressive skin and lung involvement presented a high positivity for anti-proteinase 3 antibody. Conclusion. Our study confirms the low incidence of antineutrophil cytoplasmic antibodies in scleroderma patients (3.2%); we report the first case of isolated scleroderma with positivity of anti-proteinase 3 antibodies.