Atypical presentation of pheochromocytoma as part of multiple endocrine neoplasia IIa in pregnancy

Abstract Background Pheochromocytoma in pregnancy is extremely dangerous, especially when unrecognized, and can present alone or as part of a multineoplastic syndrome. Hypertension, its hallmark, is not present in all cases, particularly in women with multiple endocrine neoplasia type IIa. Case We report a gravida with undiagnosed multiple endocrine neoplasia IIa who presented initially with peripartum cardiomyopathy but was diagnosed with an underlying pheochromocytoma. Once recognized and treated with appropriate α-adrenergic blockade, her condition reversed quickly. Conclusion Physicians should be aware that pheochromocytoma can present as cardiovascular collapse rather than just hypertension.