Tetrad presentation of non-syndromic odontogenic keratocyst: An uphill diagnostic and therapeutic challenge.

Odontogenic keratocyst (OKC), in the last decade sceptically referred to as keratocystic odontogenic tumor (KCOT), is known for its subclinical extensive growth potential and significant rate of recurrences. Odontogenic keratocyst, being the third most common cystic lesion (10-20%) of the maxillofacial region, is often recognized as a sporadic lesion and is well-documented in the literature. Multiple presentation of these cysts over a lifetime is relatively uncommon and is usually seen in conjunction with nevoid basal cell carcinoma syndrome (NBCC), orofacial digital syndrome, Noonan syndrome, Ehlers-Danlos syndrome, Simpson-Golabi-Behmel syndrome, or other syndromes.