ABSTRACTOdontogenic keratocyst is a cyst of odontogenic origin that exhibits a keratinized epithelial lining. Odontogenic keratocyst has been the topic of numerous investigators and is known for its potentially aggressive behavior and significant rate of recurrences.1OKC cyst often occurs as a solitary lesion at the angle of the mandible, however in some instances, multiple such cysts may occur in association with a syndrome called “Nevoid basal cell syndrome”.1These patients have propensity to develop multiple neoplasm including basal cell carcinoma and medulloblastoma. Hence early diagnosis and treatment is of utmost importance in reducing the severity of long-term sequelae of this syndrome. Here we present a case of multiple odontogenic keratocysts with nevi and narrowed ribs which has profound relevance to specialist in Oral Medicine and Radiology and Oral and Maxillofacial Surgery.