Goldenhar syndrome is also knowns as Oculo-Auriculo-Vertebral Syndrome which is a rare case. It represents hemifacial microsomia, macrostomia and cleft lip and palate (CLP) in facial characteristics. It has a wide range of multiple symptoms in ophthalmic, auricular, skeletal, vertebral and cardiac system. Though the etiology of this syndrome is not fully understood, it has shown variable characteristics which are caused by uncertain factors. The incidence of this syndrome ranges from 1 in 35,000 to 56,000. The treatment within an appropriate time is able to increase the chances of a good prognosis. In this paper, we report a case of patient with Goldenhar syndrome showing macrostomia and CLP. This patient underwent two surgeries; firstly, the primary lip and nose repair for unilateral complete lip and palate, secondly, the commissure repair of transverse facial cleft and skin excision of preauricular tag. Herein we continue to emphasize the necessity of proper surgery technique along with a timely treatment in conjunction with multidisciplinary management of systemic abnormalities.