Background: Mucormycosis is a highly lethal fungal infection especially in immunocompromised individuals. Methods: In order to review the epidemiology, diagnosis, and treatment of mucormycosis in renal transplant recipients we searched publications of mucormycosis cases in renal transplant recipients in PUBMED database up to December 2015. Results: A total of 174 cases in renal transplant recipients were included in this review. Most of the cases (76%) were male. Major underlying diseases were diabetes mellitus (43.1%). Rhinocerebral was the most common site of infection (33.3%). Rhizopus species was the most frequent fungus (59.1%) in patients with pathogen identified to species level. The mortality rates of disseminated mucormycosis (76.0%) and graft renal (55.6%) were higher than infection in other sites. The overall survival in patients received surgical debridement combined with amphotericin B/posaconazole (70.2%) was higher than those who received antifungal therapy alone (32.4%), surgery alone (36.4%) or without therapy (0%) (p < 0.001). The overall survivals in patients receiving posaconazole and lipid amphoterincin B were higher than that receiving deoxycholate formulation (92.3% and 73.4% vs 47.4%). Conclusions: Mucormycosis is a severe infection in renal transplant recipients. Surgical debridement combined with antifungals, especially liposomal amphotericin B and posaconazole, can significantly improve patient's overall survival.