Craniopharyngioma is a rare, benign brain tumor that has a bimodal age distribution and most often occurs in children. It usually appears in the sellar or parasellar region. We can distinguish the adamantinomatous type of craniopharyngioma associated with CTNNB1 gene mutation and the papillary type of craniopharyngioma, with a high prevalence of BRAF V600 gene mutation. Due to its specific localization, craniopharyngioma causes a variety of symptoms, the most common of which are headache, visual disturbances, endocrine disorders and other neurological disorders such as cognitive disorders or sleep disorders. Among the visual symptoms, the most common is a decrease in visual acuity and visual field disturbances. On the other hand, endocrinologically, panhypopituitarism or isolated pituitary hormone deficiency is usually observed. Hypothalamic obesity and metabolic syndrome are also common in these patients. The main imaging method for craniopharyngioma diagnosis is MRI but CT can also be useful. In the diagnosis of craniopharyngioma, it is important to include endocrinological and ophthalmological examination. Early detection of symptoms caused by craniopharyngioma and adequate treatment can prevent the development of irreversible consequences and achieve a better postoperative quality of life for the patient. The treatment of craniopharyngioma is most often based on surgical resection followed by radiotherapy. More and more studies show the benefit of using chemotherapeutics and targeted therapy for the treatment of these tumors. Despite the fact that the 5-year survival rate is very high, postoperative complications and recurrence are common.