Orthokeratinized odontogenic cyst (OOC) is a developmental cyst of jaw and was initially considered by the World Health Organization (1992) as the uncommon orthokeratinized variant of odontogenic keratocyst (OKC). However, studies have shown that OOC has peculiar clinicopathologic aspects when compared with other developmental odontogenic cysts, especially OKC. So orthokeratinized odontogenic cyst now stands out to be a distinct entity. Clinically, it occurs as a single cyst, shows a predilection for males and is most often found in the second to the fifth decade. Its bilateral occurrence is extremely rare. The purpose of the article is to present a rare case of bilateral OOC arising in the mandible and review the literature on bilateral occurrence of this lesion.