Objective The pathogenesis and pathological characteristics of Pkd1^{f/ f}: Cre transgenic mice were studied, and the transgenic mice were used for clinical research. Methods Diseased Pkd1^{f/ f}: Cre transgenic mice in their natural state were selected, liver and kidney tissues were collected, liver morphology under Doppler ultrasound was observed, the organ coefficient was determined, and the pathological conditions of liver and kidney tissues were observed by HE staining. Expression changes of PCNA and E-cadherin proteins in the liver and kidney were analyzed by histochemistry. Results Compared with the Pkd1^{f/ f} group, the liver weight and coefficient of the Pkd1^{f/ f}: Cre group were significantly increased (P<0. 01). There was no difference in body weight between Pkd1^{f/ f}: Cre and Pkd1^{f/ f} groups. There was no difference in the kidney weight and coefficient between Pkd1^{f/ f}: Cre and Pkd1^{f/ f} groups. ALT and AST levels related to liver functions were significantly higher in the Pkd1^{f/ f}: Cre group than in the Pkd1^{f/ f}: Cre group (P<0. 01). There was no difference in the levels of function-related factors BUN, CREA, and UA between Pkd1^{f/ f}: Cre and Pkd1^{f/ f} groups (P>0. 05). Conclusions Pkd1^{f/ f}: Cre mice develop liver cysts and disrupted liver functions, but no cysts appear in the kidneys.