Gorlin–Goltz syndrome (GGS) is generally characterized by the dysplasia of the skin, skeletal system, and connective tissue. In this paper, a 40-day-old baby presented with dermal and muscular agenesis on the right supraclavicular region and clinically diagnosed with GGS will be reported. To the best of the authors' knowledge, this is the first report of GGS in the literature accompanied by muscular agenesis and also this is the youngest patient diagnosed with GGS. Address correspondence and reprint requests to Dr Canser Yilmaz Demir MD, Assistant Professor, Yuzuncu Yil University Medical Faculty, Van, Turkey 65040; E-mail: canser23@hotmail.com Received 24 December, 2017 Accepted 12 April, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.
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