"Oligodontia" is the absence of 6 or more teeth, except the third molars. Genetic factors are important in determining hypodontia: in fact, this is an autosomal dominant trait relatively common in population. In particular, the agenesis of lateral incisors is fairly common, with autosomal dominant and variable expression inheritance. The incidence of hypodontia in primary dentition is 0.1-0.7% and there is no difference between females and males; instead, hypodontia in permanent dentition is most common in females compared with males and the incidence is 6-10% in general population. Oligodontia can interfere with the maxillofacial skeleton growth in children and adolescents. This problem must be tackled by paying attention to the physical and psychological development of the patient. A case of oligodontia in an 8-year-old-boy without 17 permanent teeth likely related to a variable expression genetic disorder is reported. The medical examination of the boy was completely negative, with the exception of the dentition. The child will be followed to value any possible maxillofacial abnormalities that might need an early therapy. The definitive therapeutic approach will be carried out, as soon as possible, using oral endo-osseous systems to allow normal masticatory and phonetic function.