We describe the imaging findings in a 13-year-old boy with nevoid basal cell carcinoma syndrome (NBCCS). The initial imaging work-up included plain radiographs and computed tomography (CT) of the facial area and mandible. CT detected large expansile cystic changes on both sides of the body and angle of the mandible. When the patient's condition worsened, magnetic resonance imaging (MRI) was performed to further characterize the nature of the lesions and to rule out intracranial anomalies. We agree with others who have published previous reports that although CT is valuable in elucidating osseous craniofacial anomalies associated with NBCCS, MRI is superior in demonstrating the internal composition and structure of the odontogenic keratocysts that are commonly seen in this syndrome.