Abnormalities of tooth number in development of the dentition are quite common; however, concomitant hypo-hyperdontia is a rare mixed numeric anomalous condition, especially when it occurs in the same dental arch and in a non-syndromic situation. The presence of this condition specifically in the mandibular anterior region is reported very infrequently. This case report presents the case of a 9 years old non-syndromic male with missing mandibular central incisors and an erupted mandibular mesiodens along with the review of literature. To ensure optimum function and aesthetics, cosmetic recontouring was performed after oral prophylaxis. This is the ninth case reported in the dental literature till date, with the two anomalies manifesting in the anterior region of the mandible. This article also discusses the review of literature of concomitant hypo-hyperdontia.