Merkel cell carcinoma (MCC) is a rare malignant neuroendocrine tumor more common in immunosuppressed old patients. It is characterized by a high frequency of local recurrence, regional nodal metastasis, distant metastasis, and low survival rate. The diagnosis of MCC is challenging due to its rarity and can be clinically mistaken for other skin cancer. We report a case of locally advanced MCC of the left groin with aggressive behavior that was finally controlled with a combined treatment and we collected data from the literature to discuss the appropriate therapeutic algorithm for the management of this uncommon skin tumor.