**Core Concept**
Autosomal recessive polycystic kidney disease (ARPKD) is a genetic disorder characterized by the development of cysts in the kidneys, leading to progressive kidney damage and potentially severe complications. ARPKD is caused by mutations in the PKHD1 gene, which encodes a protein called fibrocystin/polyductin. This protein plays a crucial role in the proper functioning of the cilia in the collecting ducts of the kidneys.

**Why the Correct Answer is Right**
ARPKD is a congenital disorder that typically presents in infancy or early childhood, with symptoms such as hypertension, renal insufficiency, and liver fibrosis. The cysts in ARPKD are typically found in the collecting ducts and medullary pyramids of the kidneys, and are lined by cuboidal epithelial cells. The accumulation of fluid within these cysts leads to kidney enlargement and impaired kidney function. The liver involvement in ARPKD is characterized by congenital hepatic fibrosis, which can progress to cirrhosis and portal hypertension.

**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because ARPKD is not characterized by the presence of cysts in the renal cortex.
**Option B:** This option is incorrect because ARPKD is a congenital disorder, not an acquired condition that develops later in life.
**Option C:** This option is incorrect because ARPKD is not typically associated with the development of kidney stones.

**Clinical Pearl / High-Yield Fact**
It's essential to note that ARPKD is often misdiagnosed as other renal disorders, such as autosomal dominant polycystic kidney disease (ADPKD). A key distinguishing feature of ARPKD is the presence of congenital hepatic fibrosis, which is not typically seen in ADPKD.

**Correct Answer:** D