Nevoid basal cell carcinoma syndrome is a rare autosomal dominant disorder with complete penetrance and variable expressivity. This syndrome is characterized by developmental anomalies such as multiple odontogenic keratocyst, skeletal abnormalities and multiple basal cell carcinomas. Considering the rarity of this syndrome, we present a 28 year old female affected by this syndrome. She had multiple odontogenic keratocysts, palmer and plantar pits, skeletal abnormalities such as bifid ribs and spina bifida. The dental clinician may be the first person to encounter and identify this syndrome, when the multiple cysts like radiolucencies are discovered on panoramic view.