Using metaphase chromosome spreads as substrate for indirect immunofluorescence technic, we observed anticentromere antibody in three of twenty-five patients affected with various clinical forms of localized scleroderma. Anticentromere antibody is generally considered a serologic marker of the CREST syndrome, a more benign subset of systemic sclerosis. However, none of the three anticentromere antibody-positive patients with localized scleroderma had Raynaud's phenomenon, acrosclerosis, or any signs or symptoms of systemic disease; on physical and laboratory examination, they showed only typical cutaneous features of localized scleroderma: two showed linear scleroderma, and one showed localized morphea. A 2-year 8-month follow-up of two patients did not disclose any clinical evidence of systemic sclerosis. The occurrence of anticentromere antibody in patients with localized scleroderma seems to offer supportive evidence that a relationship exists between localized scleroderma and systemic sclerosis.