Autosomal dominant polycystic liver disease, which can also have kidney cyst manifestations are associated with pathogenic variants in ALG5, ALG8, ALG9, SEC61B, SEC63, GANAB, and PRKCSH. 18, 19, 20, 21, 22 The kidney phenotype in these patients with non-PKD1 non-PKD2 ADPKD phenotypes are generally less severe and kidney failure is not usually observed without an additional contributor to kidney disease severity.18 The gene discussed is PRKCSH; the disease is Complex Cyst of Kidney.