Pheochromocytoma is an uncommon disorder associated with significant morbidity and mortality. When the disorder is discovered during pregnancy the risks are much greater. Malignant lesions represent only 10% of these tumors. Malignancy associated with pregnancy is even more rare. We present the fourth case of malignant pheochromocytoma and pregnancy to be reported in the literature. Surgery remains the mainstay of therapy. If the disorder is diagnosed early in pregnancy, surgical exploration should be considered after rapid medical control. If diagnosis is made later in pregnancy, medical control for the duration of the pregnancy should be attempted. Labor and vaginal delivery should be avoided because of an associated high morbidity and mortality. Cesarean section is recommended, once fetal maturity has been reached. Localization and definitive resection may be performed after delivery if exploration was not performed at the time of cesarean section. Although the prognosis of patients with malignant pheochromocytomas remains poor, there is hope of improving this prognosis in the future with use of new radiopharmaceutical agents and, possibly, new chemotherapy. Currently, aggressive surgical management not only appears to offer excellent palliation but likely prolongs survival of patients with malignant pheochromocytomas in selected cases.