Background: Patients with the rare, life-threatening congenital myopathy X-linked myotubular myopathy (XLMTM) require multidisciplinary care, which often includes invasive ventilator support, gastrostomy tube feeding, and wheelchair use. Real-world data assessing healthcare resource utilization (HCRU) in these patients are limited. Objectives: We analyzed a US medical claims database from 2016 to 2020 for individual medical codes (Healthcare Common Procedure Coding System [HCPCS], Current Procedural Terminology [CPT], and International Statistical Classification of Diseases and Related Health Problems [ICD-10]) to better understand HCRU in patients with XLMTM. Methods: A third-party tokenization software (Datavant) was used to maintain patient privacy and define a cohort of XLMTM patient tokens from a de-identified research registry dataset of diagnostically confirmed XLMTM patients and de-identified data from a genetic testing company, to provide a total of 80 patient tokens. An additional 112 patients were identified after October 2020 following the introduction of an XLMTM ICD-10 diagnosis code (G71.220). In total, 192 males with an XLMTM diagnosis were included. Results: The number of patients/year with claims increased from 120 to 154 and the number of claims per patients/year from 93 to 134 during the study period. Of the 146 patients with hospitalization claims, 80 (55%) were first hospitalized between ages 0 and 4 years. Across all patients, hospitalizations of 1-2 times, 3-9 times, and ≥10 times were 31%, 32%, and 14%, respectively. Patients received care from pulmonology (53%), pediatrics (47%), neurology (34%), and critical care (31%) specialists. The most common XLMTM-related conditions and procedures were respiratory events (82%), ventilation management (82%), feeding difficulties (81%), and feeding support (72%). The most frequent diagnostic codes were those investigating hepatobiliary abnormalities. This novel real-world analysis shows substantial disease burden in patients with XLMTM, including the need for respiratory and feeding support, multiple hospitalizations, and hepatobiliary complications. Conclusions: These data suggest the need for improved treatments and outcomes in these patients.