Background. Mandibular anomalies are frequently encountered among craniofacial dysmorphisms. They can be classified into congenital and acquired. Congenital mandibular anomalies are, usually, associated in plurimalformative syndromes. Objectives and methods. This study presents a retrospective analysis (1983-2010) of all the children with syndromic and nonsyndromic congenital mandibular anomalies investigated at the Clinic Municipal Hospital „Dr. G. Curteanu” – Oradea. The aim of study is to determine the incidence and to identify the main clinical and therapeutical aspects. Patients and results: We evaluated 3660 children with congenital anomalies during the last 26 years; of these, 76 (2%) presented mandibular anomalies: 29 cases of Robin sequence, 21 cases of microretrognathism associated in plurimalformative syndrome, 8 cases of Treacher Collins syndrome, 3 cases of Patau syndrome, 1 case of oculo-auriculo-vertebral spectrum (Goldenhar syndrome), 1 case of velo-cardio-facial spectrum (di George syndrome), 1 case of Silver Russell syndrome. The remaining 9 patients were identified as having congenital mandibular isolated anomalies not associated with other anomalies. Conclusion: congenital mandibular anomalies represent 2% of all congenital anomalies but probably the incidence is underestimated. The majority are associated in plurimalformative syndromes. The isolated anomalies are, probably, produced by unusual mechanical forces.