Introduction: Polycystic kidney disease (PKD) is the most common genetic cause of kidney failure. Characteristics PKD is progressive cystic dilation renal tubules (nephromegaly) and often ended being end-stage renal disease. The complications include arterial hypertension, cyst infections and refractory pain. Flank pain usually caused by increasing intraabdominal pressure, cyst rupture and infections. Imaging studies play an important role for the diagnostics, monitoring progression, and detection complications. Indications nephrectomy is recurrent and/or severe infection, symptomatic nephrolithiasis, recurrent and/or severe bleeding, intractable pain, suspicion of renal cancer, and space restrictions prior to transplantation. A Case Report: A female patient 49 y.o was anguish vomiting, stomach feels full even bit eat, complaining recurrent flank pain and feels a large mass in her abdomen getting bigger. Since 2017 she has undergoing hemodialysis.On plain Magnetic Resonance Imaging (MRI), both kidneys shows cystic lesion occupying parenchyma kidney filled by polycystic kidney and found multiple liver cysts. We plan performed nephrectomy with indication recurrent flank pain, prevent trauma and infection. But prior the procedure, patient had hemorrhagic stroke suspected complications of intracranial aneurysm. Discussion: The diagnosis Autosomal Dominan PKD(ADPKD) involve age onset disease, positive family history, abdomen was distended because of enlarge PKD and on MRI shows giant kidneys with multiple cysts on both sides and extrarenal manifestation (liver cysts). We chose nefrectomy because enlarge (size) of PKD, reccurent pain, recurrent urinary tract infection, and nutritional problems due to gastrointestinal disturbances (patient has weight lost). But patient died six days under treatment hemorrhagic stoke before we had surgery. Conclusion: The purpose nephrectomy in ADPKD to create space in the abdomen or to relief pressure symptoms to surrounding tissues and organs. Native nephrectomy is still a topic of debate. The optimum time is not well established especially for patients undergoing PD or HD.