Objective　To explore the clinical features and pathogenesis of acquired renal cystic disease caused by chronic hypokalemia. Methods　Clinical data of two patients with childhood renal cystic disease were collected and analyzed, and related literatures were reviewed. Results　Male patient 1 aged 16 years old was diagnosed with Dent disease (type 1) and male patient 2 aged 14 years old, was diagnosed with renal tubular acidosis (type 1). They both presented with renal cystic diseases and chronic hypokalemia without regular treatment and follow-up. Patient 1 also presented with abnormal renal function and patient 2 presented with nephrocalcinosis. There was no evidence on other heredity renal cystic diseases. Conclusions　Renal cystic diseases can be caused by chronic hypokalemia in some conditions, which should be diagnosed in early stage, treated timely and followed-up regularly. Key