Merkel cell tumor (MCT) typically occurs on the head or neck of elderly patients. A case is presented of a teenage girl with an MCT on her toe. The diagnosis was made 5 years after an ingrown nail on the toe was avulsed. During the interim 5 years, the periungual skin looked like granulation tissue and thus no biopsy was performed. This case of MCT is unique in three aspects: (a) extraordinarily young age, (b) atypical site, and (c) deceptive clinical appearance. Awareness of the possible occurrence of MCT in younger patients and in unusual locations will facilitate earlier diagnosis.