Introduction As the transgender patient population continues to increase, urologists and other providers who treat genitourinary malignancies will increasingly encounter cases of prostate cancer in transgender women. Little exists in the current literature to help summarize the challenges and opportunities which face this unique patient population. Similarly, little exists to provide guidance on how we may best diagnose, manage, and follow transgender women diagnosed with prostate cancer. We sought to review the available literature in hopes of providing a resource for providers moving forward. Materials and Methods We collaboratively reviewed the currently available literature, guidelines, and statements of best practice to compile a comprehensive review of this emerging and important topic. Results Transgender persons face numerous systemic barriers to care with well documented increased risks of suicide and poor health outcomes. Though uncommon, the diagnosis of prostate cancer in transgender women is often associated with significant disease. While many options for management remain in line with standard guidelines, the unique aspects of care in this population—prior/current hormone usage, gender-affirming surgical procedures etc.—must be considered. Surgical, radiation, and hormonal treatments all play a potential role in appropriate treatment. Longitudinal studies are currently lacking and clinical trials are often structured with exclusive language which may lead to further marginalization of this patient population. Conclusion Transgender persons will almost certainly continue to grow as a population encountered and treated by healthcare professionals. Better training and understanding are needed to ensure all healthcare needs are met as best possible. Prostate cancer represents an area in which great strides may be made to improve both diagnosis and treatment. Urologists, and others who manage urologic cancers, must take the lead to improve the care of transgender persons with genitourinary malignancies.