The orthokeratinized odontogenic cyst is a relatively rare developmental odontogenic cyst of the jaws that occurs predominantly in males between the third and fourth decades. According to the 2005 World Health Organization’s classification, orthokeratinized odontogenic cyst is not a part of the keratocystic odontogenic tumor spectrum. We present a case of a 41-year-old male with a history of remarkable lingual expansion in the anterior part of the maxilla since last year with rapid growth in the last three months as a unilocular well–defined radiolucent lesion extending from maxillary left central incisor to the right second premolar. Orthokeratinized odontogenic cyst is a specific type of keratinized odontogenic cysts which is completely different from keratocystic odontogenic tumor from the viewpoint of the clinical and pathological features.