In an eleven year old girl Atrophodermia idiopathica et progressiva Pasini et Pierini, a rare variety of localised scleroderma developed within six months as well outlined, reddish-brown, patchy lesions without induration on her right side. The histological examination revealed perivascular inflammatory infiltrations in all corium layers, and discrete sclerosis radiating into fat tissue cells. The levels of antinuclear antibodies and C-reactive protein were both elevated. During intravenous therapy with penicillin G the skin alterations faded but did not disappear.