Congenitally missing teeth (CMT) are among one of the commonly known dental anomalies. The most frequently missing teeth in the permanent dentition, excluding the third molars, are mandibular second premolars and maxillary lateral incisors. Exclusive agenesis of both maxillary canines is an extremely rare occurrence and only a few cases have been reported. Previous studies showed that the prevalence of maxillary canine agenesis varies between 0.07 and 0.13%. In recent studies on Indian population, no cases of maxillary canine agenesis have been documented. This paper reports a case of non-syndromic bilateral agenesis of permanent maxillary canines, along with agenesis of both mandibular central incisors in a healthy 13-year-old Indian female patient; and a brief literature review on prevalence, etiology and treatment modalities of the condition. How to cite this article: Kambalimath HV, Jain S, Patil RU, Asokan A, Kambalimath D. Permanent Maxillary Canine Agenesis: A Rare Case Report. Int J Clin Pediatr Dent 2015; 8(3):242-246.