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Objective: The objective of the review is to investigate the experiences of patients with lysosomal storage disorders treated with enzyme replacement therapy and the experiences of their families. Introduction: Lysosomal storage disorders are rare diseases caused by mutations in genes encoding proteins required for lysosomal function. The onset of lysosomal storage disorders vary from infancy to adulthood, depending on the specific disease and disease type. Enzyme replacement therapy is the standard treatment for some lysosomal storage disorders. The adherence to the treatment by patients and their families is affected not only by the symptoms but also by the scheduling of hospital visits. No qualitative systematic review on this topic has examined the experiences of patients and their families. Inclusion criteria: Qualitative studies dealing with the experiences of patients with lysosomal storage disorders receiving enzyme replacement therapy and their families will be included. These may include experiences related to satisfaction or dissatisfaction with being diagnosed, difficulties and expectations continuing treatment, the advantages or disadvantages related to school and work life, and the psychological burden and supports on families. This review will consider studies in all settings because relevant experiences of patients and their families may occur outside medical institutions. Methods: MEDLINE, CINAHL Plus, APA PsycINFO, Scopus, and Igaku Chuo Zasshi databases will be examined from 1991 to the present. Studies published in any language will be included. The study selection, critical appraisal, data extraction, and data synthesis will be performed in accordance with the guidelines for systematic reviews of qualitative evidence in the JBI Manual for Evidence Synthesis. Systematic review registration number: PROSPERO CRD42019147751