Abstract Pheochromocytomas are rare catecholamine-secreting tumors that occur in 0.002% of pregnancies. These tumors result in high maternal and fetal morbidity and mortality unless diagnosed in early stages of development, because excess levels of catecholamines cause vasoconstriction of both maternal and uteroplacental vasculature. Paroxysmal hypertension is the most common manifestation, but its variability in presentation and similarity to other pregnancy-related conditions often make diagnosis of pheochromocytoma difficult. Thus, it is essential to consider underlying pathological causes of hypertension during gestation. Diagnosis and treatment of pheochromocytoma must be approached uniquely given the physiologic changes during pregnancy. The standard of care for diagnostic imaging during pregnancy is with magnetic resonance imaging. For these reasons, knowledge of therapy for pheochromocytomas in the pregnant patient is essential for clinical endocrinology practice.