Although the presence of pheochromocytoma in pregnancy is extremely rare, this association deserves much attention as the tumor constitutes a very high risk for both mother and fetus. Any pregnant woman with hypertension, especially if paroxysmal or labile, or with so far unexplained "spells", should induce the clinician to consider the possibility of a pheochromocytoma. Maternal and fetal survival depend a lot on an early diagnosis, a correct medical therapy and a correct timing of delivery and surgery. In this respect, a strict collaboration between obstetricians, endocrinologists, anesthesiologists and surgeons is pivotal.