Adenoma malignum (AM) is a rare variant of cervical adenocarcinoma with an unfavorable prognosis despite radiation therapy, surgery, or chemotherapy either alone or in combination. Hitherto, however, the effectiveness of hormonal therapy for this condition has not been evaluated. We report on a patient with cervical AM treated with progesterone before surgery. The progesterone therapy resulted in a complete clinical response and partial surgical response. Later on the treatment was changed to tamoxifen because of side effects of the progesterone treatment. The patient is still without evidence of disease 42 months after the start of the hormonal therapy. The progesterone receptor analysis on the biopsy was clearly positive. This is, to our knowledge, the first case of an AM responsive to hormonal treatment. Furthermore, this is the first case of an AM with bone metastases at the time of primary diagnosis.