The authors present a case of a Sertoli-cell type androblastoma of the ovary. The tumor is mainly seen in young women and is mostly discovered through hormonal dysfunctions linked to it. Histopathological evaluation is indispensable for an exact diagnosis. Because of its rarity, there is little experience with the tumor and the histological identification is also sometimes very challenging. The tumor is occasionally associated with Peutz-Jeghers syndrome, tumor of the thyroid, or goiter. Biologically it follows a low-malignant course with a fair prognosis, long-term complex patient follow up is necessary after unilateral oophorectomy of the diseased organ.