Simple SummaryRhabdomyosarcomas are soft tissue tumors more commonly found in children than adults. The diagnosis and management of these tumors continues to change as our knowledge of how they behave and how they respond to previously used treatments has improved. We treat these tumors differently based on where they develop. Rhabdomyosarcomas of the genitourinary tract are the second most common, arising from the bladder, prostate, vagina/uterus, and paratesticular regions. Advancements in treatment options, including chemotherapy and radiation, have led to improved outcomes and have allowed, in many instances, for less drastic surgical resections. In this review, we discuss how to approach rhabdomyosarcomas in general, with an emphasis on the presentation, diagnosis, treatment, and outcomes of those arising from the different organs in the genitourinary tract.AbstractRhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric and adolescent population, with 350 new cases diagnosed each year. While they can develop anywhere in the body, the genitourinary tract is the second most common primary location for an RMS to develop. Overall survival has improved through the increased use of protocols and multidisciplinary approaches. However, the guidelines for management continue to change as systemic and radiation therapeutics advance. Given the relative rarity of this disease compared to other non-solid childhood malignancies, healthcare providers not directly managing RMS may not be familiar with their presentation and updated management. This review aims to provide foundational knowledge of the management of RMSs with an emphasis on specific management paradigms for those arising from the genitourinary tract. The genitourinary tract is the second most common location for an RMS to develop but varies greatly in symptomology and survival depending on the organ of origin. As the clinical understanding of these tumors advances, treatment paradigms have evolved. Herein, we describe the breadth of presentations for genitourinary RMSs with diagnostic and treatment management considerations, incorporating the most recently available guidelines and societal consensus recommendations.