Focal dermal hypoplasia or Goltz syndrome is a rare X-linked genodermatosis characterized by cutaneous and musculoskeletal defects. Dermoscopy is a noninvasive auxiliary method for the diagnosis of lesions, whether melanocytic or not. Its widespread use in dermatology is resulting in the description of new patterns and characterization of lesions not reported before its use. A typical case of Goltz syndrome presenting multiple malformations was observed and submitted to dermoscopy. Dermoscopy findings of the papillomas in raspberry form in the perioral and ocular regions, revealed a unique vascular pattern, different from viral warts; dermoscopy of some brownish maculas resembling lentigo in the periphery of skin atrophic areas are described as lentigo-like lesions, an uncommon pattern of melanocytic lesions, but without criteria suggestive of malignancy.

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