Pheochromocytomas and paragangliomas (PPGL) are a rare type of neuroendocrine tumors, arising from chromaffin cells of the adrenal medulla and sympathetic ganglia. The clinical presentation is based on episodic or continuous hyperproduction of catecholamines, which can be manifested by hypertensive crises and/or persistent arterial hypertension. The symptoms are mostly non-specific (hypertension, diaphoresis, palpitations, etc.) and can easily be attributed to other conditions - the reason why pheochromocytoma was called the "great imitator". Some patients are asymptomatic. Despite this, the presence of pheochromocytoma can have cardiovascular, cerebrovascular and many other negative consequences. It can also be presented as a life-threatening condition with multiorgan failure. After the initial suspicion of PPGL, it is necessary to carry out biochemical tests with the aim of determining the concentration of metanephrines and their metabolites in plasma or urine. In addition, a detailed family history is taken and the potential presence of a hereditary syndrome associated with pheochromocytoma is investigated. As many as 10-25% of cases are thought to be hereditary; most often within the syndrome: MEN-2, neurofibromatosis and von Hippel Lindau. Thanks to the development of genetic testing, many mutations have been successfully proven. Therefore, in recent literature, it is described as one of the most common hereditary tumors. The therapy of choice is laparoscopic adrenalectomy. Considering the risk of arterial pressure fluctuation, it is necessary to introduce blockers of α adrenergic receptors (and β if necessary). Currently, many targeted therapies are in the research phase. Long-term follow-up is necessary due to the risk of recurrence and metastasis.