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The isolated autricle-facial spectrum is a defect that can present in a unique way or as a complex and heterogeneous disease; from the dysmorphological point of view it is classified as a disruption. These defects, which usually occur in the way of the first brachial arch, have the minimum form, which are the pre-auricular appendages, and the most severe, presenting macrostomia and epibulbar dermoid, which is known as Goldenhar syndrome and may be associated with other malformations. It is presented a girl with a microtía grade IV, light facial asymmetry that was presented in isolation, with no apparent cause that caused this alteration. The interrogation and the physical examination are considered of great value to arrive at the clinical diagnosis and to be able to realize an adequate genetic advice.