We report on a young woman with pheochromocytoma associated with pregnancy and review 41 other cases reported in the literature from 1988 to 1997. This review reveals that the overall maternal mortality was 4 percent and the fetal loss 11 percent; antenatal diagnosis of pheochromocytoma reduced maternal mortality to 2 percent; however, fetal loss was 14 percent. Diagnosis of pheochromocytoma was made antepartum in 83 percent of the cases. Although pheochromocytoma associated with pregnancy is rare, a high index of clinical suspicion must be kept and all those at risk must be investigated to achieve an early diagnosis and improved outcome. Once the diagnosis is confirmed, alpha-adrenergic blockade is essential and beta-blockade may be required. Magnetic resonance imaging and computerized tomography scan may be used to localize the tumor during the antenatal period. In early pregnancy, i.e., before 24 weeks, both tumor resection and medical treatment are associated with good fetal outcome; in later pregnancy, elective cesarean delivery followed by tumor resection results in favorable maternal and fetal outcome. BACKGROUND Obstetricians & Gynecologists, Family Physicians. OBJECTIVE After completion of this article, the reader will be able to understand the clinical manifestations of a pheochromocytoma during pregnancy, how to make the diagnosis of a pheochromocytoma during pregnancy, and to know the medical and surgical management of a pheochromocytoma during pregnancy.