Background: Limb-girdle muscular dystrophy type 2I/R9 (LGMD2I/R9) is a rare neuromuscular disorder caused by FKRP variants impairing α-dystroglycan glycosylation. From 10/2022-10/2025, ICD-10-CM code G71.038 (“other LGMD”) was used in administrative claims to identify patients (pts) with LGMD2I/R9 and related autosomal recessive subtypes. Few studies report healthcare resource utilization (HCRU); none report costs, indicating a literature gap. This study describes the clinical and economic burden of pts identified by code G71.038 vs matched controls. Methods: This retrospective study used Optum Market Clarity® electronic health records (EHR)/administrative claims data. Pts had ≥2 records in EHR and/or claims with ICD-10-CM code G71.038 on separate days 10/1/2022-9/30/2024 (index date=first record). Outcomes (demographic/clinical characteristics, treatment patterns, HCRU, and medical costs) were evaluated before/after index. A 10:1 control cohort was matched on age, sex, index month/year, geographic region, and payer. Results: Of the 521 pts with other LGMD, 375 were eligible for HCRU/cost analyses (median follow-up 10.6 months); the matched cohort included 5210 in the full cohort, 4093 in the HCRU cohort. Mean age was 53.6 years; 68.5% were female. During follow-up, cardiac (29.0%) and pulmonary (39.2%) involvement exceeded that in controls (16.5% and 21.9%). In the 6 months before/after index, 26.5% of LGMD pts were nonambulatory/had assisted ambulation, 15.0% had ventilation support, and 3.8% had ventilation dependence. During follow-up, 40.3% received corticosteroids and 30.1% heart failure medications, (controls 19.6% and 25.8%, respectively). In the HCRU cohort, 13.3% had ≥1 inpatient (IP) stay, 29.6% ≥1 emergency department (ED) visit, and 98.4% ≥1 outpatient visit, compared with 6.6%, 16.4%, and 95.7% of controls. IP and ED rates were higher for nonambulatory/assisted ambulation pts (IP 27.0%; ED 48.0%) vs ambulatory pts (IP 8.4%; ED 22.9 %). Total medical costs per pt per year were substantially higher vs controls ($41,200 vs $13,919); costs were higher for assisted ambulation ($48,417) and nonambulatory ($125,727) pts vs ambulatory pts ($33,995). Conclusions: Findings show considerable clinical burden and high HCRU and costs for pts with LGMD2I/R9 and related subtypes, with consistently higher comorbidity and medical costs than matched controls. The new ICD-10-CM code for LGMD2I/R9 will enhance research and characterization of this pt population.