A 62-year-old female who developed concomitantly acrosclerosis and keloid-like lesions is described. Biopsy specimens from these linear lesions showed a fibrous proliferation in the dermis composed mostly of normal-appearing, horizontally oriented collagen bundles and myofibroblasts, thus resembling a scar. Keloidal scleroderma is a rare variant of scleroderma. The diagnosis is considered for patients with scleroderma who develop lesions clinically and histologically indistinguishable from keloids. This case demonstrates for the first time that the histopathologic findings can mimic those of a scar, rather than a keloid, and hence is appropriately designated as keloid-like scleroderma. Clinical pathological correlation is mandatory for the correct diagnosis.