Syringomatous adenoma of the nipple (SAN) is an extremely rare disease originating in the adnexal gland; it was first reported by Rosen in 1983 [Am J Surg Pathol 1983;7:739-745]. Since then, 34 cases have been reported worldwide. We present the case of a 51-year-old man with SAN, in whom local excision of the tumor was performed. Histologically, the tumor consisted of tubules, ductules and epithelial cell strands, and most of the proliferating ducts presented with a characteristic teardrop or comma-shaped appearance.