ObjectiveThe aim of the study was to investigate the clinical characteristics, therapy strategies and prognosis of Sertoli‐Leydig cell tumors (SLCTs).MethodsA total of 50 cases of ovarian SLCTs were retrospectively analyzed. We performed descriptive statistics to describe baseline characteristics.ResultsA total of 70% of SLCT patients were below the age of 45 years. The dominant pathologic types were poor (40%) and moderate (40%) differentiation. Retiform variant tumor is more aggressive and tends to appear in children. According to the 2014 FIGO (the International Federation of Gynecology and Obstetrics) classification, tumors were classified as Stage I (n = 46: well differentiated, n = 4; moderately differentiated, n = 19; poorly differentiated, n = 18; and retiform, n = 5), Stage II (n = 1, moderately differentiated), Stage III (n = 1, poorly differentiated), and Stage IV (n = 2: poorly differentiated, n = 1; and retiform, n = 1). Median follow‐up was 58.1 months (2–132 months). A total of 45 patients (90%) achieved clinical remission. Four patients experienced a relapse (34.5 months, range: 7–58) and one patient died of the tumor at 10 months after initial treatment. All patients in grossly Stage IA achieved complete response, irrespective of the presence or absence of staging surgery or chemotherapy. After treatment, five patients successfully gave birth to healthy babies.ConclusionThe prognosis for women diagnosed with early stage (I–II) SLCT is generally favorable, especially Stage IA. while those with advanced stages, poorly differentiated, retiform variant, or tumors containing heterologous components exhibit a more aggressive clinical course. Fertility sparing surgery appears to represent a feasible treatment approach for early stage SLCTs.