Merkel cell carcinoma (MCC) is a rare but aggressive skin cancer associated with the Merkel cell Polyomavirus. Its incidence and mortality are increasing. There have been many advances in the last several decades in the etiology, detection, and management of MCC, but much about its natural history and most effective treatment remains unknown. Surgical excision with margins of 1 to 2 cm remains first-line therapy for early-stage MCC, but robust evidence supporting immunotherapy for patients with advanced disease has led to recent approval of immune checkpoint inhibitors in the treatment of advanced MCC.