Idiopathic atrophoderma of Pasini and Pierini (IAPP) was first described by Italian dermatologist Agostino Pasini (1924) and Argentinean dermatologist Luis E. Pierini (1936). The disease usually manifests by brownish spots of various size and number mainly on the torso. Thus far the issue has been discussed whether atrophoderma is a non-sclerotic atrophic variant of morphea or is an individual nosological entity. The cases when the same patient simultaneously had localized scleroderma, lichen sclerosus et atrophicus and atrophoderma in different parts of the body are described. This suggests a possible similarity in pathogenesis of these diseases. The histological pattern of IAPP does not have characteristic features along with a large external similarity to localized scleroderma. Atrophoderma can exist for more than 10 years without complications and transformation into systemic form. Thus, the clinical picture is monomorphic (spots) without improvement regardless of the treatment, frequently mild atrophy is noted. The clinical observation of a 17-year-old patient suffering from extensive IAPP for 8 years is presented. During this time, the patient was repeatedly treated in hospitals, received therapy as in the case of localized scleroderma. Against this background, the pathological process progressed in the form of an increase in the lesions’ area of the previous pigmented spots and the appearance of new ones without signs of inflammation and compaction, assuming the extensive nature of process. There is no clear answer to the question whether this is a variant of localized scleroderma or an independent disease. In this observation we prefer to consider extensive idiopathic atrophoderma of Pasini and Pierini as independent nosology.