Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant inherited condition that causes lifelong progressive muscle weakness. Despite the significant disease burden, little has been published on the costs associated with a diagnosis of FSHD to the healthcare and payor systems. Most previous studies examining FSHD-associated costs were conducted outside of the United States. To better understand healthcare utilization and cost associated with FSHD in the United States, we conducted a retrospective case-matched control analysis of OptumLabs’ deidentified administrative claims data from Medicare Advantage and commercially insured health plan enrollees. The primary aim of the analysis was to estimate annualized medical claims cost for FSHD patients and compare these costs to controls. Secondary aims of the analysis included identifying subgroups (e.g., age, gender, ambulation) that may drive higher medical claims costs and identifying any comorbidities that may disproportionately affect FSHD patients. We report here results from the analysis, which included 383 FSHD patients and 1915 matched controls. FSHD patients with commercial insurance had significantly higher medical claims costs than controls. An accompanying study examined direct and indirect costs to patients and families with FSHD in the US.