Dental anomalies of tooth number influencing the development of permanent dentition are quite common, and more numeorus than in the case of primary dentition, however, combined occurrence of hypodontia and hyperdontia is a rare phenomenon, especially in the same dental arch. The purpose of the present report is to describe a case of concomitant hypo-hyperdontia (CHH) in an 8 year-old male with missing mandibular central incisor and an erupted mandibular mesiodens, and its minimally invasive management. This is a unique case of CHH in the mandible in the dental literature, the two anomalies being manifested in the anterior region of the mandible. Furthermore, this is the only case report describing the evidence of taurodontism in association with mandibular CHH.