The Merkel cell carcinoma is a rare tumor of the subdermal tissue. Its origin are Merkel cells or the subcutaneous pluripotential stem cells. The etiology of the tumor is unknown, however, exposition to UV radiation and immunosuppression are relevant predisposing factors. Clinically, a subcutaneous rapidly growing tumor with or without enlarged lymph nodes is seen. Beside excisional biopsy and various imaging modalities, somatostatin-receptor-scintigraphy plays an important role in diagnostic. Surgical resection of the tumor combined with neck dissection followed by radiation therapy is the current therapy of choice. Different chemotherapy protocols are available for patients with distant metastasis.