The syndrome of unilateral anotia, and ipsilateral facial palsy is one of the extremely rare manifestations of genetic abnormalities.The occurrence of microtia/anotia syndrome in association with congenital facial palsy has been reported in association with teratogenic effect of thalidomide and retinoic acid, well-Known syndromes such as Berry Treacher Collins and Goldenhar, and poorly characterized cardiofacial syndrome or syndromes including the cases of Matthies, Pearl, Hall, Bretton Chappuis and Engel, and Girisha and Phadke.There have been very few reports of the occurrence of microtia/anotia syndrome in association with congenital facial palsy without other congenital cardiac defects or other congenital abnormalities. These reports include the reports of Parkash and colleagues, Gathwala and colleagues and Mahale, et al.The aim of this paper is to describe the extremely rare occurrence of the syndrome of congenital ipsilateral facial palsy and unilateral anotia in an Iraqi infant. This Iraqi patient most probably represents the fourth patient with the syndrome of microtia/anotia syndrome and congenital facial palsy without other abnormalities.