A 17-year-old boy underwent a wide partial mastectomy with axillary dissection for secretory breast cancer, followed by low grade irradiation (30 Gy). There were no axillary lymph node metastases. Hormone receptor assay of the tumor was positive for progesterone and negative for estrogen. There was no local recurrence or sign of dissemination after nearly 5 years. Literature search revealed 21 cases of secretory breast cancer in childhood and adolescence; in only 2 cases was the hormonal status studied. We report an additional case and review the literature.