ARPKD accounts for approximately 2-3% of the polycystic disease affecting the kidneys, with 75 to 80 percent of cases manifesting in the neonatal period [3]. PKHD1 gene has a complicated transcription profile and undergoes complex alternative splicing that generates multiple variants of the fibrocystin/polyductin (FC/PD) protein [4, 5]. This evidence concerns the gene PKHD1 and autosomal recessive polycystic kidney disease.