This study was conducted to investigate the association between symmetrical agenesis of the mandibular third molars and agenesis of other teeth in Japanese orthodontic patients. A sample of 64 patients with symmetrical agenesis of the mandibular third molars and without agenesis of the maxillary third molars was selected as the third molar agenesis group. In addition, 585 patients with all 4 third molars were selected as controls. The main materials examined for diagnosis of tooth agenesis were panoramic radiographs. The prevalence rate of tooth agenesis was compared between the groups using the chi-squared or Fisher's test, and the odds ratio. Significantly increased occurrence of overall agenesis of the maxillary lateral incisors, and overall and symmetrical agenesis of other teeth including the maxillary and mandibular second premolars and mandibular first molars were observed in the patients with symmetrical agenesis of the mandibular third molars and without agenesis of the maxillary third molars. Irrespective of the presence or absence of symmetrical agenesis of the mandibular third molars, no significant differences in the prevalence rates of overall or symmetrical agenesis of other teeth and specific teeth were noted between the sexes or between the right and left sides of the jaws in the patients without maxillary third molar agenesis. (J Oral Sci 58, 171-176, 2016).