Background: Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal dominant syndrome that has various expressions in each patient. Generally; NBCCS is followed by multiple nevoid basal cell carcinoma of the skin, orbital anomalies, skeletal anomalies, central nervous system anomalies and multiple odontogenic keratocysts (OK). NBCCS is usually diagnosed between the ages of 5-30 years, with multiple basal cell carcinomas of the skin and OKs in the jaws as the initial findings.
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