
Gorlin-Goltz syndrome, a rare inherited syndrome occuring in an autosomal dominant manner, presents with basal cell carcinomas, odontogenic keratocysts (OKCs), and skeletal and dermal anomalies. This case report describes occurrence of this syndrome in a 12-year-old female patient, causing distortion of lower jaw. Prompt identification and intervention are vital to mitigate the potential for grave complications, such as malignancies of the skin and brain, and significant disfigurement or destruction of the oral and maxillofacial region due to cysts in the jaw.