Autosomal recessive polycystic kidney disease (ARPKD) is a genetic disorder that primarily affects the kidneys and liver. It is caused by mutations in the PKHD1 gene, leading to the formation of numerous fluid-filled cysts in the kidneys. These cysts can disrupt normal kidney function, resulting in complications such as high blood pressure and kidney failure. ARPKD typically presents in infancy or early childhood, but symptoms can vary widely. In addition to kidney issues, affected individuals may experience liver problems, including portal hypertension. Early diagnosis and management are crucial for improving outcomes and quality of life for those with this condition.