Pure Sertoli cell tumor (SCT) is a rare sex cord tumor and a subtype of Sertoli-Leydig cell tumors according to the WHO Classification. They lack a Leydig cell component and do not contain the immature neoplastic stroma found in the neoplasms of the Sertoli-Leydig cell category. The age of the patients ranges between two and 79 years. Sertoli cell tumors occur in women of reproductive age but a few can also occur in children. The most common clinical presentation when occurring in children is isosexual pseudoprecocity. Women of reproductive age and postmenopausal women frequently present with abdominal pain, swelling and menstrual abnormalities. Occasionally SCTs occur in patients who have Peutz-Jeghers syndrome. The tumors are hormone functional in 40-60% of cases. They are often estrogenic, occasionally also androgenic or rarely both. Grossly they are usually yellow to brownish, solid or with several cystic areas. Microscopically they show always almost a tubular growth pattern, but they may also have other growth patterns which can be extensive, making the correct diagnosis difficult. These histologic patterns may result in SCTs mimicking other ovarian tumors. The immunohistochemical panel which usually includes EMA, inhibin, chromogranine, CD99 and calretinin is often helpful in establishing the diagnosis. Most SCTs are Stage I, unilateral, cytologically bland, and clinically benign, but occasional examples are high stage. About 11% of Stage I tumors have worrisome histologic features that may portend an adverse outcome.