Background: and Purpose: The Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS) is a reliable and valid instrument for evaluating the functional status of patients with spinal and bulbar muscular atrophy (SBMA). This study aimed to validate a Korean version of the SBMAFRS in an SBMA population. Methods: We applied the SBMAFRS to 64 SBMA patients at their regular follow-up clinical visits. The patients underwent clinical evaluations that included the 6-minute walking test (6MWT), forced vital capacity (FVC), manual muscle test, and the Penetration-Aspiration Scale (PAS). To estimate the stability of the SBMAFRS, the scale was reapplied to a subset of 31 randomly selected patients within 4 weeks of the initial test. The convergent validity was evaluated, and correlations were examined between SBMAFRS items and the muscular force, the total and subscores on the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), FVC, PAS score, age at onset, disease duration, and 6MWT results. Results: The internal consistency of the scale was confirmed by a high Cronbach’s alpha (total raw alpha=0.867, total standardized alpha=0.863). The test-retest reliability as assessed by Spearman’s rho was also high. The total score and subscores of the SBMAFRS were strongly correlated with the respective items and subscores of the ALSFRS-R, respiratory function, and the 6MWT. Conclusions We have performed a validation study of the Korean version of a disease-specific functional rating scale for SBMA patients. The SBMAFRS is a useful tool for clinical practice and as a potential outcome measure for Korean SBMA patients.