Supernumerary teeth are those teeth present in addition to the normal number of teeth and are most frequently seen in the maxillary incisors and molar regions. In the molar regions, supernumerary teeth are divided into two types depending on their location: distomolars and paramolars. Distomolars usually occur in the form of a fourth molar distal to the third molar. Distomolars are a developmental anomaly and have been argued to arise from a combination of genetic and environmental factors. Therefore, distomolar management should be part of a comprehensive treatment plan. This article presents a case report of an unusual occurrence of a rare unilateral distomolar in the right of the maxilla with multiple abnormalities in a 21-year old healthy subject. In addition, a mini literature review includes the definition, types, prevalence, classification, etiology, complications, diagnosis, and therapeutic strategies, which may be adopted in the occurrence of distomolars.