The deletion of both genes leads to the development ofsevere renal cystic disease, which shares similar radiological and morphological features with ADPKD [9].In a study by Sampson et al., constitutional deletions of TSC2 and PKD1 genes were linked to severe renal cystic disease, which resembledthe clinical presentation of ADPKD. The gene discussed is PKD1; the disease is cystic kidney disease.