Pheochromocytoma is a rare catecholamins producing tumor developing from chromaphine tissue of adrenal medulla or extraadrenal tissue (paraganglioma). In the last years knowledge of genetics of the disease and diagnostics and treatment of pheochromocytoma has grown. About 24 % of pheochromocytomas are inborn. They are associated with MRN2 syndrome, von Recklinghausen´s neurofibromatosis of type 1, von Hippel Lindau´s syndrome and mutation of genes coding subunits B,C and D of enzyme of succinatdehydrogenasis. The basis of hormonal diagnostics is to determine plasma-free nephrines which have 99 % sensitivity. From imaging methods CT or MRI are standard methods and from functional imaging it is MIBG scan, octreo scan and PET which is important in diagnostics of malignant pheochromocytoma. The author analyses therapeutic procedures in treatment of benign and malignant pheochromocytoma.