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Background: Pompe disease is a glycogen storage disease primarily affecting striated muscles. Despite its main manifestation in muscles, patients with Pompe disease may exhibit non-muscle symptoms, such as hearing loss, suggesting potential involvement of sensory organs or the nervous system due to glycogen accumulation., Aims: This study aimed to evaluate the presence of concomitant small and large fiber neuropathy in patients with Pompe disease., Methods: In this case series study, nine patients with Pompe disease without complaints of neuropathy were evaluated. Small fiber neuropathy was assessed using the Small Fiber Neuropathy Screening List (SFNSL) and SUDOSCAN R , while the sympathetic system was evaluated through Sympathetic Skin Response (SSR), and large fiber neuropathy was assessed through electrodiagnostic findings., Results: Small fiber neuropathy was detected in seven patients (77.8%) according to the SFNSL. Three patients (3/9, approximately 30%) exhibited positive electrophysiological tests, including SSR, SUDOSCAN R , and nerve conduction studies for neuropathy. They also had positive SFNSL results., Conclusions: This study indicates that neuropathy can be a comorbid condition in Pompe disease, emphasizing the importance of screening for this disabling condition., Competing Interests: Declarations. Ethics approval and consent to participate: This study was conducted in accordance with the principles outlined in the Declaration of Helsinki and was approved by the Research Ethics Committee of Shahid Beheshti University of Medical Sciences (approval number: IR.SBMU.MSP.REC.1400.068). Informed consent was obtained from parents and patients, and the patient’s privacy and confidentiality were strictly maintained throughout the study. Consent for publication: We did not use any identifying images or other personal or clinical details of participants that could compromise their anonymity. Therefore, this item is not applicable to this study. We confirm that all authors listed in the manuscript are aware of the submission and have agreed to submit the paper to this journal. Competing interests: The authors declare no competing interests., (© 2024. The Author(s).)