Secretory breast carcinoma (SBC) is a rare tumor that was originally described in children and adolescent women, with a characteristic morphology and controversy regarding the choice of treatment. This unusual breast cancer subtype generally has a favorable prognosis, although several cases have been described in adults with increased tumor aggressiveness and a risk of metastases. Surgery is considered the most appropriate treatment for this pathology. The present study describes the case of a 12-year-old female who presented with a painless lump in the left breast, and subsequently underwent a biopsy of the sentinel lymph node and a partial resection of the left breast (breast-conserving therapy). Periodic follow-up examinations after completion of the surgical and chemotherapeutic treatment have shown no evidence of either local regression or distant metastases and, one year later, the patient remains free of the disease. This study suggests that local excision with sentinel lymph node mapping may be a suitable therapeutic approach for children with SBC.