Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare hereditary autosomal dominant condition, with high penetrance and variable expressivity. The aim of this study is to report 2 cases diagnosed at emergency hospital units with the specialty of maxillofacial surgery and traumatology. The first refers to a 10-year-old patient who sought consultation due to an expressive increase in the maxilla volume with panoramic radiography evidencing multiple unilocular radiolucent images in the gnathic bones. The second is a 20-year-old woman presenting extraoral increased volume in the mandibular region with signs of infection; imaging exams showed 2 unilocular radiolucent images in the mandible. After clinical, radiological, and histopathologic evaluation, multiple keratocysts, skeletal changes, and other criteria that characterize the syndrome were identified. In both cases, surgical treatment of the odontogenic cysts was performed, and the patients remain in multiprofessional follow-up.