Craniopharyngioma is a tumor of benign histology, although it can cause serious sequelae due to its location and growth in the sellar and suprasellar region. It is characterized by being aggressive due to its ability to invade locally, and by its high recurrence rate. Histologically, two distinct tumors have been acknowledged, which were previously considered subtypes. The adamantinomatous craniopharyngioma is the most frequent in children and is caused by an activating mutation of the CTNNB1 gene, which codes for a mutant form of beta- catenin. Whereas, papillary craniopharyngioma develops almost exclusively in adults, and it is associated with mutations in the BRAF oncogene and activation of the MAPK signaling pathway. The symptoms of both of these tumor types are secondary to hormonal deficits, hydrocephalus or visual disturbances. Treatment should be performed in centers with experience in them, achieving a balance between a wide resection and protection of hypothalamic function. Radiotherapy has proven to reduce tumor recurrence, with a current focus on the development of medical treatments based on molecular targets.