Moebius syndrome is characterized by sixth and seventh nerve palsy. Involvement of other cranial nerves is also common. Occasionally, cranial nerves V, X, XI and XII are involved, resulting in difficulty of chewing, swallowing, and coughing, often leads to respiratory complications. Other variable features include orofacial dysmorphism and limb malformations. Mental retardation and autism have been reported in some cases. Most cases of Moebius syndrome are sporadic, but familial occurrence has been reported. In 1996, the incidence of Moebius syndrome in Dutch population was estimated to be 0.002%. Approximately one tenth of the patients wi th Moebius syndrome were reported to present Poland anomaly. We here report a 3 year old Turkish girl with Moebius syndrome who also has right cerebellar hemisphere and posterior vermis hypoplasia. Her parents are first cousins. At birth she was noted to have facial asymmetry which is due to right 6 th and 7 th cranial nerve palsy. She had psycohomotor retardation, developed gait ataxia and abnormal EEG pattern. Cytogenetic analysis revealed normal karyotype. Harbord et al. (1989) reported a case of Moebius syndrome with unilateral cerebellar hypoplasia to have right facial weakness, complete paralysis of the right lateral rectus muscle and partial paralysis of the left, and slight ataxia of the right arm. Although the co-occurence of unilateral cerebellar hypoplasia with Moebius syndrome in both cases would be considered as a co-incidence, it would also be speculated that unilateral cerebellar hypoplasia is a rare finding of Moebius syndrome.