On abdominal computed tomography scan for evaluation of hematuria, the index patient, a 23-year-old male, was found to have multiple renal cysts bilaterally. He had been on peritoneal dialysis for 6 years, complicated by intermittent episodes of exit-site infection and peritonitis. Over time, he developed recurrent urinary tract infection and nephrolithiasis with intermittent hematuria. His maintenance dose of recombinant erythropoietin was discontinued, and he maintained a normal hemoglobin level. Annual surveillance of the bilateral renal cysts with magnetic resonance imaging revealed development of a complex cystic mass that warranted further investigation. Following a urology consultation, laparoscopic nephrectomy was performed without complications and with minimal interruption of PD. Pathology evaluation revealed renal cell carcinoma within the renal parenchyma and multiple cystic structures consistent with acquired cystic kidney disease. No clear recommendations exist in the literature regarding continuation or interruption of PD in the perioperative period for planned nephrectomy, tumor resection, or other uncomplicated abdominal or retroperitoneal procedures. A perioperative management strategy for PD patients requiring abdominal or retroperitoneal surgery is outlined here. If these recommendations are followed, patients may continue PD with minimal interruption, preservation of the peritoneal membrane, and few complications.