Objective: To investigate the clinical and genetic characteristics and long-term prognosis of Fabry disease patients with kidney involvement. Methods: This single-center retrospective case series study included 78 Fabry patients with kidney injury diagnosed from January 2003 to September 2025. Demographic data, multi-system involvement, kidney biopsy, biochemical indicators, gene sequencing results, and follow-up data were analyzed. Results: Males accounted for 70.5% and classical type for 41.0%. Male patients had earlier onset/diagnosis, lower α-galactosidase A activity and higher Lyso-GL-3 levels (all P<0.05). The proportion of cardiac involvement was higher in males than in females, but the difference did not reach statistical significance (P = 0.116). Coexisting pathologies (mostly IgA nephropathy,19.2%) were found in 30.8% of biopsied cases. 49 GLA gene variants were identified, 8 of which were novel. Truncating mutations were associated with earlier onset, classical phenotype and higher Lyso-GL-3, but not with more severe organ involvement. During a median follow-up of 3.42 years, median renal survival after diagnosis was 12.7 years. Male sex predicted worse renal survival, while mutation type did not. Conclusion: This study delineates the clinical and genetic spectrum of Chinese Fabry patients with kidney injury, reporting 8 novel GLA variants, and highlights sex differences, substantial individual variability among female patients, high frequency of coexisting nephropathy, and limited predictive value of GLA genotype for long-term renal outcomes.