<p>Rare malignant tumors of the nail unit, fingers, and toes pose diagnostic and therapeutic challenges due to their varied presentations, functional implications, and limited treatment data. While Mohs micrographic surgery (MMS) is established for common skin cancers, its role in rare acral malignancies remains unclear. A systematic review was conducted in April 2025 examining PubMed, Embase, and Cochrane databases to review outcomes of MMS for rare malignant tumors of the digits, excluding melanoma, basal cell carcinoma (BCC), and squamous cell carcinoma (SCC). The included studies reported MMS treatment for histologically confirmed rare acral malignancies with follow-up on recurrence or functional preservation were extracted. There were 17 studies (18 patients) included. Tumors treated with MMS included digital papillary adenocarcinoma, dermatofibrosarcoma protuberans, Kaposi sarcoma, malignant onychopapilloma, acral myxoinflammatory fibroblastic sarcoma, apocrine adenocarcinoma, and sarcomatoid pilomatrix carcinoma. All cases achieved histologic margin clearance with MMS. There were no recurrences reported (follow-up: 0.75–60 months). Functional preservation was reported in 17 of 18 patients: two required partial amputation due to tumor extent. MMS appears to be an effective, tissue and digit-sparing treatment for rare malignant tumors of the nail unit and digits. Further studies are needed to confirm long-term outcomes.</p>