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Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor that usually occurs in the proximal extremities and trunk. The purpose of this systematic review was to assess patient demographics, treatment approach, and outcomes for patients with LGFMS of the distal upper extremity. Methods: We performed a literature review of case reports and patient reports of LGFMS using Medical Literature Analysis and Retrieval System Online (MEDLINE) and PubMed. Inclusion criteria included case reports and case series of patients with LGFMS of the distal upper extremity. Animal studies, comments, and non-English publications were excluded. Data on available patient demographics, treatment regimen, recurrence, and metastasis were collected. Results: A total of 365 publications were reviewed. After applying the inclusion and exclusion criteria, 19 articles were included. There were 24 unique patients with LGFMS of the distal upper extremity. The median age was 32 years, and most patients were treated with a surgical excision. Of the 15 reported outcomes, 4 patients had local recurrences, and no patient had metastases or died due to the tumor. Conclusions: Although LGFMS of the distal upper extremity is less common, it is critical for surgeons to consider it as a differential. The current approach is a wide excision with negative margins, and chemotherapy or radiotherapy may not be necessary. We found decreased proportions of local recurrence and metastases in the distal upper extremity compared with other anatomical regions. We recommend that surgeons follow up with their patients indefinitely given the slow mitotic rate of LGFMS.