Noel Taboada LugoAizar Ríos AyalaNoelya Velky Montecinos Zubieta2026-03-222026-03-222018https://andeanlibrary.org/handle/123456789/66986Klingmuller made the first description of two brothers with this dysmorphic pattern in 1956 and it was Giedion who, a decade later, used the name tricho-rhino-phalangeal for this syndrome. Phenotypically, three types are described with different clinical gradations. Type I or Giedion syndrome is the one that shows the highest incidence worldwide, has a lower severity in the clinical phenotype. It is transmitted with an autosomal dominant inheritance pattern. The case of a 10-year-old patient is reported. The patient had a dysmorphic pattern and typical radiological findings that led to the clinical diagnosis of this syndrome. A detailed delineation of the clinical and behavioral phenotype of the patient is conducted, as well as the differential diagnosis with other genetic syndromes with a similar dysmorphic pattern.esMedicineDifferential diagnosisPediatricsIncidence (geometry)Gynecologyarticle