Introduction: Congenital syndromic or non-syndromic absence of maxillary permanent canine teeth is a rare occurrence. This anomaly can adversely affect esthetics and masticatory function. Other dental anomalies that can be associated with tooth agenesis include tooth malposition, insufficient bone height, and periodontal disease. According to the statistics, the incidence of canine agenesis rang-es from 0-4.7% with a prevalence rate of 3.0%. Non-syndromic canine agenesis combined with other types of tooth agenesis, like mandibular central incisor agenesis, has been occasionally described in the literature.
Case Presentation: This case report presents a nine-year-old male patient with three canine agenesis combined with both mandibular central incisors and unilateral maxillary lateral incisor agenesis. Results: Tooth agenesis is a common dental anomaly causing significant esthetic im-pairment. Its proper management requires early detection and a multidisciplinary approach.