Introduction: Gorlin-Goltz or nevoid basal cell carcinoma syndrome was already described in the 19th century. The cause for this autosomal dominant inherited disorder is a mutation in the PTCH1 gene (9q22.3) for the patched receptor, a negative regulator of the effects of sonic hedgehog. Gorlin Goltz is characterized by development of basal cell carcinoma in the third decade of life and keratocystic odontogenic tumors in the second and third decade of life. For specialists in the field of dentistry, especially cystic lesions may give a hint to Gorlin Goltz. Histopathologically, keratocystic odontogenic tumors consist of multiple or solitary cavities surrounded by fibrous capsule and lined with keratinized epithelium. Within the fi-brous stroma, typically, single daughter cysts are identified which are infiltrating the sur-rounding bone and causing the high recurrence rate. Case report: A 17-year-old male patient was referred to our Clinic for Oral and Maxillofacial Surgery of the Münster University Hospital by a Dentist with suspected odontogenic cyst on the right side of the upper jaw. About five years earlier, the patient underwent multiple keratocystic odontogenic tumor removal and Gorlin-Goltz syndrome was suspected, however there was no further evaluation. Panoramic tomography image showed well-defined cyst-like translu-cency with sclerotic rim. Teeth numbers 13 - 16 depicted clear signs of tooth displacement. In addition, radiography of the paranasal sinus was done which showed calcification of the falx cerebri. Under general anesthesia, cystectomy with curettage of the bone cavity and peripheral ostectomy was performed. The histopathologic report confirmed the suspicion of keratocystic odontogenic tumor. Summary: With a prevalence of 1-9/100,000, Gorlin-Goltz syndromes rank among the Rare Diseases as defined by the European Union. According to Evans clinical diagnosis is based on the presence of two major or one major and two minor criteria. Since keratocystic odontogenic tumors as typical manifestation of Gorlin-Goltz syndrome are revealed in the specialty of the dental clinician, he/she may be the first to raise the suspicion of the syndrome in as yet undiagnosed cases. Keratocystic odontogenic tumors have high recurrence rates because of infiltrative growing daughter cysts, thorough follow-up is required in case of positive histopathological findings. Keywords: rare diseases, Gorlin syndrome, keratocystic odontogenic tumor