Eleven variants (c.7866C > A, c.7960A > G, c.7979A > T, c.7987C > T, c.11248C > G, c.11251 C > T, c.11257C > G, c.11257C > T, c.11346C > T, and c.11393C > G distributed in PKD1 and c.1480G > T located in PKD2) that were previously described as missense, synonymous, or nonsense variants in ADPKD patients were confirmed to be pathogenic by affecting pre-mRNA splicing. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.