Hemifacial macrosomia (HFM) is the second most common facial congenital anomaly. Deficiency of hard and soft tissue on one side of the face is its obvious clinical finding, which can cause facial asymmetry. Hemifacial microsomia is described in three grades of severity, although grade II has two subgroups (mild and severe). Many anomalies can be misdiagnosed with different grades of Hemifacial microsomia, e.g. Treacher Collins syndrome, traumatic postnatal deformity, Goldenhar syndrome, hemi mandibular elongation, Parry-Romberg syndrome, juvenile rheumatoid arthritis, Nager acrofacial dysostosis syndrome, post axial acrofacial dysostosis, muscle dysfunction, branchio-oto-renal syndrome and Maxillofacial dysostosis. The first step to treating patients with HFM, is an accurate diagnosis. Decision making for treatment planning of patients with HFM, is highly dependent on the severity of the deformity and patients age. In mild grades of the anomaly, functional therapy can improve facial and occlusal symmetry when they are young, but in more severe grades, imposition of orthopedic treatment, may be undesirable and waste of time. Early surgical interventions to encourage the growth in the affected condyle may be helpful in severe cases; however, consultation with the surgeon is advised to determine the patients who need early surgery. Distraction osteogenesis is a controversial treatment modality, which still needs more long-term studies.