Six patients suffering from ovarian sex cord tumor with annular tubules (SCTAT) were reported in this article with special reference to the clinical features, histological characteristics, sex hormone profile, and management of disease. SCTAT was documented to be a estrogen-progesterone-secreting tumor based on the observations of glandular atrophy and decidual change of stroma in the endometrium and assays of steroid hormone. Menometrorrhagia followed by persistent amenorrhea and pelvic mass were presented as important clinical features. This tumor was considered as a tumor with low-grade malignancy, and retroperitoneal lymphatic metastasis was thought to be an important pathway of spread. Unilateral salpingo-oophorectomy together with ipsilateral pelvic and para-aortic lymphadenectomy were suggested as an effective treatment for SCTAT. Radiotherapy can be used for local recurrence and distant metastases.