Background <p>Merkel cell carcinoma (MCC) of the hand is a rare and aggressive malignancy characterized by early regional and systemic spread. In the hand, surgical management is particularly challenging because achieving adequate excision margins may compromise function.</p> Methods <p>A systematic review was conducted according to PRISMA guidelines. PubMed/MEDLINE and Scopus were searched for studies reporting primary MCC of the hand or digits. Patient-level data were extracted, including demographics, tumor characteristics, surgical management, adjuvant treatments and oncologic outcomes.</p> Results <p>Twenty-nine studies including 55 patients were analyzed. Mean age at diagnosis was 68 years, with male predominance. Immunosuppression was reported in 39% of cases. Digital involvement was most frequent, and dorsal localization predominated. Limb-preserving surgery was performed in 77% of patients, whereas amputation was performed in 23%. Amputations were more commonly associated with tumors involving dorsal and periarticular structures of the proximal ulnar digits; however, comparable anatomical patterns were also observed in cases treated with limb-preserving approaches. Disease progression occurred in 49% of patients and was predominantly metachronous. Local recurrence was uncommon, occurring in 6% of cases, and was observed after both wide local excision and amputation.</p> Conclusions <p>MCC of the hand demonstrates aggressive systemic behavior, with prognosis primarily driven by metastatic spread rather than local disease control. The low rate of local recurrence observed after both limb-preserving surgery and amputation suggests that no clear oncologic superiority of digit-sacrificing procedures can currently be established. Surgical management should therefore be individualized within a multidisciplinary setting, balancing oncologic safety with preservation of hand function whenever feasible.</p> <p> Level of Evidence: not gradable.</p>