Recurrence of focal segmental glomerulosclerosis (FSGS) is a major challenge in kidney transplantation. Several clinical factors, including initial steroid sensitivity, have been associated with increased post-transplant FSGS recurrence risk. However, conflicting data have been reported, possibly due to the heterogeneous pathophysiology of FSGS and the lack of genetic testing of FSGS patients. Further, the response to immunosuppressive therapies have not been evaluated. This study aimed to assess the risk factors for post-transplant recurrence in stringently selected patients based on a comprehensive clinicopathological evaluation and genetic testing. Fifty-nine patients aged 1–25 years at FSGS onset who underwent kidney transplantation between 2002 and 2018 were enrolled. Patients with secondary, familial, syndromic, and genetic FSGS and those who did not undergo genetic testing were excluded. Data from 15 kidney transplant recipients were analyzed. Nine (60%) patients experienced post-transplant FSGS recurrence, while six patients did not. The proportion of patients who achieved complete or partial remission with initial steroid and/or additional therapies with immunosuppressive agents and/or plasmapheresis was significantly higher in the FSGS recurrence group than the group without FSGS recurrence (P=0.04). In conclusion, this study suggests that the response to steroid treatment, other immunosuppressive agents, and/or plasmapheresis may predict post-transplant FSGS recurrence.