Gorlin—Goltz syndrome is a rare disease inherited by the autosomal dominant type. It is characterized by various manifestations, including multiple foci of basal cell carcinoma of the skin, keratocysts of the bones of the facial skull, skeletal abnormalities, manifestations of the cardiovascular system and an increased risk of developing tumors of the brain, heart, kidneys, etc. The article considers a clinical case of a patient with identified Gorlin—Goltz syndrome, manifested by multiple foci of basal cell carcinoma and keratocysts of the mandible. The article describes the surgical treatment of a patient with an extensive decaying tumor of the frontal-parietal region, which grows into the bones of the skull and dura mater. The clinical case illustrates the possibilities of a multidisciplinary approach to the treatment of cancer patients with seemingly inoperable tumor processes. A reasonable combination of classical oncology, neurosurgery and reconstructive surgery in patients with widespread tumor processes involving structures of different structure and function allows for a good result.