Focal dermal hypoplasia (FDH) or Goltz syndrome is a rare genetic skin disorder; characterized by abnormalities of both ectodermal and mesodermal structures. We report the case of a girl of 8 years of Moroccan origin, second of a fratery of two, resulting from a non-consanguineous marriage and without any particular family history. Examination at admission notes that the patient presents with an overall hypotrophy, right facial asymmetry with low-set protruding ear, narrow nasal bridge. The examination of the oral cavity notes: Arborescent papillomas of the oral mucosa, hypertrophy gingival, abnormous tooth form, ectopic extensive dental caries eruption. Skeletal abnormalities: Ectrodactyly ("lobsterclaw" hand deformity) of the right hand, Syndactyly bilateral second and third toes. Hypopigmentation of the abdomen skin, microphthalmia of the right eye. The rest of the clinical examination is without particularity. The genetic study confirmed the diagnosis of FDH by highlighting a mutation of the PORCN gene.