Polycystic Kidney Disease (PKD) is an inherited kidney disorder characterized by the formation of multiple fluid-filled cysts in the kidneys, frequently involving other organs such as the liver. It is a progressive condition that can lead to end-stage renal disease (ESRD). This case report aims to explore the clinical course and management approach of a PKD patient with poor follow-up adherence since diagnosis. A 55-year-old female with a diagnosis of PKD since 2009 presented with abdominal enlargement, fatigue, and right upper quadrant discomfort. Despite a positive family history, the patient did not receive regular monitoring and relied on herbal remedies. Over 16 years, disease progression was marked by significant increases in serum urea and creatinine, development of multiple renal and hepatic cysts, and progression to chronic kidney disease (CKD) stage 4–5. The patient currently suffers from hepatic cyst complications and ESRD. This case highlights the accelerated progression of PKD in the absence of consistent clinical monitoring and early therapeutic intervention. It underscores the importance of early detection, regular renal function evaluation, patient and family education, and supportive care. Planning for renal replacement therapy and genetic screening of offspring are crucial to mitigate disease progression and improve outcomes in PKD patients.
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