Syringoacanthoma is a rare tumor derived from the eccrine acrosyringium; very few cases have been reported. The nosology of this tumor remains controversial. We present a new case of syringoacanthoma occurring in a 43-year-old woman; the lesion lasted for more than 30 years and caused partial destruction of the earlobe. Histopathologic examination showed a hyperkeratotic intraepidermal tumor, with many large cavities lined by an eosinophilic cuticle and filled with a PAS-positive substance. This case is probably a mutilating variant of syringoacanthoma, despite benign histopathologic features.