Pheochromocytoma and paraganglioma (PPGL) are rare tumors derived from the adrenal medulla and extra-adrenal chromaffin cells. Timely diagnosis is necessary due to the dangerous effects of excess catecholamines on the cardiovascular system and also for the potentially aggressive biological behavior of PPGL. Diagnosis may be challenging due to the great variability in clinical presentation, even if nowadays PPGLs are often diagnosed incidentally due to the incidental finding of adrenal or retroperitoneal masses or thanks to surveillance in carriers of mutation in PPGL susceptibility genes. A biochemical diagnosis cannot be omitted before proceeding to the imaging study and it relies on plasma or urinary free metanephrine and normetanephrine testing. Appropriate preoperative evaluation is essential as PPGL surgery has a high risk of intraoperative hemodynamic lability. The alpha-adrenergic blockers doxazosin or phenoxybenzamine are the treatment of choice, and fluid replacement is often necessary to restore preoperative volume depletion and to treat postoperative hypotension. Distinguishing a benign PPGL from tumor with metastatic potential is very challenging: the size of the primary tumor, invasion of surrounding tissues, lymph node metastasis are all clinical predictors of metastasis, but also the molecular and biological characteristics are considered to predict the risk of malignancy.