Introduction:Acute graft-versus-host-disease (GVHD) in kidney recipients is extremely rare. Knowledge about its clinical manifestations, diagnosis, treatment, and prognosis is limited and needs to be increased.Clinical Findings:One male kidney transplant recipient developed diarrhea and suffered kidney function damage. Primarily diagnosed with acute rejection, he was given methylprednisolone (MP) bolus treatment. Meanwhile, intravenous immunoglobulin (IVIG) and decreased immunosuppressive agents were applied for the corresponding infection. During the treatment, skin rashes occurred over his whole body. Biopsies were then taken. The pathology of the kidney graft showed no rejection, while the skin pathology revealed typical GVHD. Furthermore, fluorescence in situ hybridization proved the presence of donor-derived cells in the skin lesions, and infiltrating cytotoxic T cells and NK cells were identified in the rash.Outcome:Based on the clinical presentations, pathological findings, and chimerism detection, GVHD after kidney transplantation was confirmed as the final diagnosis. The recipient responded well to treatment. His kidney function recovered, and the skin lesions were completely resolved. He has been followed for 1 year without any further episodes.Conclusion:GVHD after kidney transplantation has its own characteristics. In the presence of a highly immunocompromised state, diarrhea and rashes, a diagnosis of GVHD needs to be considered. Kidney function impairment may be involved. Pathological changes and detection of chimerism and immunocyte infiltration are required for diagnosis. MP bolus, IVIG, and decreased immunosuppression could be beneficial to the clinical outcome. Kidney recipients have a prognosis superior to recipients of organs bearing large numbers of lymphocytes.