Abstract
Gorlin–Goltz syndrome (nevoid basal cell carcinoma syndrome) is a rare autosomal dominant disorder caused due to mutation in the PTCH (patched gene) located in the arm of the ninth chromosome. The disease is characterized by increased incidence for nevoid basal cell carcinoma and other multi-organ disorders including multiple odontogenic keratocysts. Early diagnosis and stringent follow-up are necessary for preventing untoward consequences of the disease. This case report emphasizes on the necessity of adhering to proposed criteria and need for thorough investigation to rule out central nervous system lesions in the patients.
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