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Myoepithelioma is an exceptionally rare tumor, primarily arising in glandular tissues but occasionally found in soft tissues, including the hand. Its occurrence in the hand is particularly uncommon, presenting unique clinical challenges due to the limited number of documented cases and the unusual location. We conducted a literature review in June 2024, with the aim to evaluate the current understanding of hand myoepithelioma, recent diagnostic advances, treatment options, and the diverse presentations of this neoplasm. Articles confirmed that patients present with a painless, slow-growing mass in the hand, often misdiagnosed as more common soft tissue tumors like lipomas or fibromas. Imaging, particularly MRI and ultrasound, aids in assessing the tumor, but definitive diagnosis relies on histopathology, including immunophenotyping. Managing spindle cell myoepithelioma in the hand requires a multidisciplinary approach, with surgical excision being the primary treatment. Achieving clear margins is critical yet challenging due to the hand’s complex anatomy. In some cases, adjuvant therapies such as radiation or chemotherapy may be necessary. The prognosis depends on factors like tumor size, location, and the success of surgical removal, with complete excision typically leading to a favorable outcome.