Key Clinical Message (63 words):

A 70-year-old female with autosomal dominant polycystic kidney disease (ADPKD) and hypertension developed profound intraoperative hypotension during bilateral nephrectomy, subsequently attributed to an undiagnosed peri-renal paraganglioma. This case underscores the importance of comprehensive preoperative evaluation in ADPKD patients with severe hypertension. Greater awareness and early detection of rare tumours like pheochromocytoma or paragangliomas (PPGLs) are essential to improving surgical outcomes and patient safety.