Abstract: A 41 years old female patient experienced enlarged right nipple more than 6 years ago. Physical examination showed enlarged and hardened right nipple (about 2 cm) but no tenderness. Mammographic results showed the mixed calcification pattern, which was presenting multiple sizes of circular and granular calcification of the nipple areola complex. Ultrasound revealed large calcification in the nipple areola area with posterior acoustic shadow. This patient underwent extensive excision of the nipple areola complex. The surgical margin was confirmed to be negative. Postoperative pathology confirmed an infiltrating syringomatous adenoma with ossification in some areas. Tumor cell infiltration was not found at the surgical margin. After 4 years of follow-up, no recurrence was found. The mammography and ultrasound findings of this benign tumor were significantly different from those reported in previous literatures. We should improve the understanding of the variousness of clinical imaging manifestations of this disease.