**Core Concept**
Rokitansky-Kuster-Hauser (RKH) syndrome, also known as Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a rare congenital disorder characterized by the absence or underdevelopment of the vagina and uterus in individuals with a normal 46,XX karyotype. This condition is often associated with renal anomalies and other pelvic malformations.

**Why the Correct Answer is Right**
RKH syndrome is caused by mutations in genes involved in the development of the Müllerian ducts, such as WNT4 and RSPO1. These genes play a crucial role in the formation of the uterus, fallopian tubes, and upper part of the vagina. Individuals with RKH syndrome typically have a normal karyotype and are phenotypically female, but they may experience reproductive tract anomalies, including vaginal agenesis, unicornuate uterus, and renal abnormalities. The characteristic features of RKH syndrome include vaginal agenesis, unicornuate uterus, renal anomalies, and other pelvic malformations.

**Why Each Wrong Option is Incorrect**
**Option A:** This option is a characteristic feature of RKH syndrome, so it is not the correct answer.
**Option B:** This option is also a characteristic feature of RKH syndrome, so it is not the correct answer.
**Option D:** This option is not a characteristic feature of RKH syndrome, but it is a feature of other conditions, such as Mayer-Rokitansky-Küster-Hauser syndrome with obstructed hemivagina and ipsilateral renal anomaly (MOH).

**Clinical Pearl / High-Yield Fact**
RKH syndrome is often associated with renal anomalies, including renal agenesis, horseshoe kidney, and other pelvic malformations. A high index of suspicion and a thorough physical examination are essential for diagnosing this condition.

**Correct Answer:** D.