Abstract
Axenfeld Rieger's syndrome (ARS) is a rare disease causing genome abnormalities, impeding the development of the alveolar process due to disproportionate jaw growth. This case report presents the interdisciplinary management of an 8-year-old child with ARS exhibiting caries, malformed teeth (conical/microdont teeth), infraoccluded primary molars and hypodontia. After liaising with his paediatrician, treatment was carried out under local anaesthesia which included fissure sealants, fillings, upper overdenture and lower resin-bonded bridge. For aesthetics, an upper overdenture was made to correct occlusion and upper lip support, and also fixed resin-bonded bridge on the lower anterior segment. Early diagnosis and an interdisciplinary approach are necessary to provide the best short- and long-term treatment plans, as well as treatment and follow-up for individuals with the syndrome.