Background: Carney complex is a rare, autosomal-dominant, multisystem disorder characterized by endocrine overactivity, spotty skin pigmentation, and myxomas.
Case: We present the case of a 24-year-old primigravid woman with a pregnancy complicated by Carney complex. At 18 weeks of gestation, severe hypertension developed. Medical history was significant for chronic hypertension, nephrolithiasis, and an atrial myxoma excised in 2011. She had Cushingoid features, an elevated 24-hour urine free cortisol, and a cutaneous myxoma. At 26 weeks of gestation, superimposed preeclampsia developed. She underwent a primary classical cesarean delivery, delivering a live female weighing 650 g.
Conclusion: Carney complex is a rare cause of hypercortisolism and hypertension during pregnancy. It should be considered when features of Cushing syndrome and severe hypertension are present.