Abstract: Adamantinomatous craniopharyngioma (ACP) is a rare, benign, but locally aggressive brain tumor derived from the embryonic remnants of Rathke’s pouch. Although its incidence is bimodal, primarily affecting children and adults, the adamantinomatous variant predominates in children and poses significant diagnostic and therapeutic challenges due to its proximity to critical structures, such as the optic chiasm and hypothalamus. These tumors are characterized by mutations in the CTNNB1 gene, leading to activation of the WNT/β-catenin pathway, and clinically present with visual disturbances, signs of intracranial hypertension, and endocrine dysfunction. Typical MRI findings include lobulated, predominantly cystic masses with calcifications and gadolinium enhancement.