Purpose: Fibromuscular dysplasia (FMD) is a non-atherosclerotic systemic arterial disease that is not infrequently discovered during kidney donor evaluation. They can be diagnosed pre-operatively in living donors, intra-operatively in deceased donors, or post-operatively in deceased donors. Due to chronic shortage of kidney donors, allografts with FMD have been used in renal transplantation. We review articles with renal allografts with FMD and the techniques used to treat renal artery stenosis. Methodology: A literature review was performed in MedLine, PubMed, and Embase for search terms “fibromuscular dysplasia”, “kidney transplantation” or “renal transplantation”, and “renal artery stenosis”. All relevant articles were included. Results: 19 studies were included and reviewed. There were 17 case reports and 2 case series. Management of FMD in renal allografts were either prophylactically performed or for therapeutic purposes. Prophylactic techniques described include resection of affected segment +/- reconstruction with venous graft or arterial graft. Symptomatic FMD in renal allografts present as graft dysfunction such as hypertensive crisis or rising creatinine. Therapeutic techniques described include open surgical revision and endovascular treatment of renal artery stenosis. Majority of studies described stable allograft function post intervention, albeit short-term outcomes. Conclusion: Diagnosis of FMD in donor kidneys is not a contraindication for transplantation. There are several described techniques to prophylactic manage FMD in donor kidneys or to treat symptomatic renal artery stenosis in donor kidneys with FMD. However, little is known about the long-term outcomes of these renal allografts and further studies are warranted.