Merkel cell carcinoma (MCC) is a rare, malignant skin cancer, exhibiting neuroendocrine differentiation, with a significant incidence of locoregional lymph nodal involvement (40%-73%). The accepted staging system classifies MCC as: stage I, localized skin disease; stage II, regional lymph node disease; stage III, metastatic disease. The clinical differentiation of stage I and II patients is difficult and understaging is frequent. Surgery, as first approach, represents the leading treatment for this neoplasm and, depending on stage consists in: local wide excision for stage I patients and local excision and lymphadenectomy for stage II. In our experience, lymphadenectomy, included in the initial treatment of all stage II patients, seemed to influence positively the prognosis. In comparing stage related recurrence and survival rates the results we obtained were better in stage II patients, where lymphadenectomy was included in the initial treatment than in stage I subjects, who received local excision alone as first treatment and lymphadenectomy as secondary treatment for nodal recurrence (overall recurrence rate 86% vs 20%, survival rate 71% vs 80% in stage I vs stage II patients). The performance of lymphadenectomy for stage I MCC could be reconsidered both for a more reliable staging of the disease and for a positive impact on recurrence and survival rates.