Background: Adamantinoma is a rare lesion of low-grade malignancy, and represents 1% of malignant bone tumours of bones, and is mainly located in two regions of the body, jaw (ameloblastoma), and lower extremities. The treatment of choice is surgery due to it being a radio- and chemotherapy-resistant neoplasia.
Clinical Case: A 39 year old male with a history of neonatal hydrocephalus with moderate psychomotor retardation.