A dentigerous cyst is a developmental odontogenic cyst occurring most commonly in the third molar region of mandible and maxilla and maxillary canine region followed by mandibular premolar areas. However, dentigerous cyst occurring in association with impacted maxillary premolars is a very rare presentation accounting for less than 0.5% of total dentigerous cyst cases. In the following case report, a dentigerous cyst was reported in maxillary premolar region in a nonsyndromic patient having one missing premolar and another malformed one associated with the dentigerous cyst. How to cite this article: Jain N, Gaur G, Chaturvedy V, Verma A. Dentigerous Cyst associated with Impacted Maxillary Premolar: A Rare Site Occurrence and a Rare Coincidence. Int J Clin Pediatr Dent 2018;11(1):50-52.