BACKGROUND Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor of dermal origin. Treatment recommendations are limited owing to a paucity of retrospective data and an absence of prospective data. The objective of this study was to determine current therapeutic trends and their impact upon outcome. METHODS A retrospective study (1983 to 1996) was performed with patients from the Department of Defense and our University-affiliated hospitals. RESULTS Thirty-five patients were evaluated with a mean follow-up of 31 months. Overall, 1- and 2-year survival rates were 80% and 50%, respectively. Patients undergoing wide local excision, prophylactic lymph node dissection, and adjuvant radiotherapy had significantly decreased locoregional and distant recurrence rates and improved survival when compared with their counterparts. Adjuvant chemotherapy did not diminish recurrence rates nor improve survival. Both locoregional and distant recurrence significantly decreased survival. CONCLUSIONS These data suggest that early aggressive treatment for MCC improves both tumor control and survival, whereas the early use of chemotherapy does not improve outcome.