At the age of 51 years, several kidney cysts were detected, raising the suspicion of polycystic kidney disease, which was later excluded by next-generation sequencing genetic testing: negative for Polycystin 1 (PKD1), Polycystin 2 (PKD2), and Ciliary IPT domain-containing fibrocystin (PKHD1) genes. Here, PKD1 is linked to Complex Cyst of Kidney.