| Summary: | Background : Hemifacial microsomia or Goldenhar syndrome is a congenital abnormality that it's main features are an one-sided under development of ear (or Artesia), jaw and neck.
This syndrome is associated with additional anomalies and from view point of phenotype is highly variable.
Case Report: The case was a one day old girl born to a 30 years old woman by normal vaginal delivery.
Congenital abnormalities were noted on physical examination and included right microtia with ear canal Artesia ,hypertelorism ,micrognatia ,defect between the right ear and lip and cardiac murmur.
Conclusion: Goldenhar Syndrome is a congenital abnormally which manly affects face, but another organs involvement should be considered .Cardiac problems are the main causes of death in these patients.
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