Various symptoms may be present in pheochromocytoma and paraganglioma. The tumor can imitate numbers of diseases and often leads to misdiagnosis. Current advances in laboratory techniques (determination of free plasma metanephrines), tumor localization (using positron emission tomography) and surgery techniques together with better understanding of pathophysiology and genetics (role of subunit B of succinate dehydrogenase) lead to earlier diagnosis, changes in strategy and treatment options. Authors introduce case report of retroperitoneal paraganglioma in 59 years old patient. In conclusion, interdisciplinary cooperation in diagnosis and treatment was successful.