Hamadan University of Medical Sciences Avicenna Journal of Dental Research 2423-7582 15 4 2023 12 29 Major Manifestations of Gorlin Syndrome in a Patient With Hemophilia A 183 188 10.34172/ajdr.1675 EN Elham Hadadian Pour https://orcid.org/0009-0006-5573-0248 Amirhosein Haghir Sirous Risbaf Fakour https://orcid.org/0000-0002-6006-1943 Journal Article 10.34172/ajdr.1675 2022 12 25 2023 07 11 Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominantly inherited condition in which constitutive pathway activity and tumor cell proliferation are disturbed due to defects in hedgehog signaling. This study reports an adult case of hemophilia A suspected of having Gorlin syndrome. There is no similar evidence in the literature to describe the association between these disorders. NBCCS is characterized by the development of multiple basal cell carcinomas (BCCs). The case presented in this study had two major manifestations of NBCCS, including the calcification of the falx cerebri and the presence of keratocystic odontogenic tumors (KOTs); however, no minor manifestations of NBCCS were found in this case. On the other hand, given the financial restrictions, it was impossible to perform a genetic examination to confirm the presence of NBCCS.