Agenesis described as a certain developmental disorder that is a lacking in the number of teeth present intraorally. Hypodontia is an agenesis with the absence of one or several teeth. In most of the population, the prevalence is 3,5 - 6,5%, with 0,3-0,4% for a severe hypodontia (an absence of 6 teeth or more clinically). Agenesis affects permanent dentition more often than primary dentition, and the incidence of agenesis is greater in female than male patients. Agenesis might be a result of enviromental and genetic factors. It is necessary to have a comprehensive treatment for a case of hypodontia, which involves restorative, orthodontic, a prosthetic treatment based on the children's growth and development. In the present case, a patient of 6 years and 5 months old girl was diagnosed with severe hypodontia. The treatment of choice included a removable ortodontic appliance for the upper jaw and removable space maintainer for the lower jaw. The removable orthodontic appliance served as an interceptive orthodontic treatment to correct anterior crossbites, and the child will be refered to the orthodontist to treat a Class III skeletal malocclusion. The space maintainer was to be used until the patient's condition become favorable for a permanent fixed bridge or steel removable partial denture.