The atlas (C1) is known to show congenital anomalies in its anterior and posterior arches. Though posterior arch anomalies are well known, the anterior arch anomalies are seldom reported in the literature [1]. Currarino et al. [1] have classified posterior arch anomalies into five types: Type A, failure of fusion of two hemi arches; Type B, defect in one side of the arch, Type C, defects on both sides of arch, Type D, absence of arch except posterior tubercle, and Type E, absence of whole arch including tubercle. Type A is the frequent type and it occurs in 5.4% of the population and 97% of all posterior arch defects. When compared to posterior arch, the occurrence of anterior arch anomalies is rare [2]. So far only few cases of anterior arch of C1 including either absence or clefts have been reported [3].Anomalies of C1 may cause atlantoaxial joint instability which necessitates the treatment with a cervical collar or surgery. Furthermore, these anomalies may increase the