HNF1B and autosomal dominant polycystic kidney disease: We present a case mimicking autosomal dominant polycystic kidney disease (ADPKD), highlighting the phenotypic heterogeneity of <i>HNF1B</i>-related disease.<h4>Presenting concerns of the patient</h4>A 37-year-old man presented with hypertensive urgency, accompanied by flank pain and abdominal distension.