Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a sexual developmental disorder. In this disorder, there is a congenital absence of the uterus and vagina with normal external genitalia. The etiology is not well understood. Variations of this condition exist that may include congenital abnormalities and psychological problems. In this article, we discuss the case of a 47-year-old African American female who presented with acute renal failure, solitary right kidney, and a pelvic mass extending from the pelvis to the right hypochondrium determined to be a fibroid. The patient was managed by a multidisciplinary team, dialyzed, and planned for removal of the mass. While understanding the low probability of having fibroids without a uterus, fibroids should not be excluded from such patients. It is also important to consider the emotional and psychological well-being of such patients.