Eccrine nevus (EN) is a very rare hamartoma of the skin and with varying clinical manifestations. Histologically, these neoplasms present as a proliferation of normally structured eccrine sweat glands in the dermis. There have been no more than 20 cases previously have been reported in the English literature. Herein we report a 25-year-old man with eccrine nevus on the neck. To our best knowledge, this is the first case that the lesion affecting the neck