Pheochromocytoma during pregnancy is unusual, with approximately 250 cases now reported in the literature. The current case describes the anesthetic management of a patient with a large extra-adrenal pheochromocytoma who underwent a combined cesarean section and tumor removal in which perioperative hemodynamic control was difficult despite high-dose a-adrenergic blockade. In this case it is believed that difficulty in perioperative hemodynamic management was related to mechanical stimulation of the tumor by the gravid uterus. Review of published case reports since 1980 reveals that a pheochromocytoma during pregnancy may be diagnosed in an extra-adrenal location relatively more commonly compared to non-pregnant patients. Established criteria exist for the adequacy of alpha- and beta-adrebergic blockade for the management of pheochromocytoma in the non-pregnant patient. We discuss how these criteria apply to pregnant patients with pheochromocytoma, and suggest possible modifications to the criteria.