Objectives Sertoli Leydig cell tumors (SLCT) belong to the group of sex cord-stromal tumors. SLCTs of the ovary are rare (less than 0.5% of all ovarian tumors). The aim of this study is to establish the epidemiological and prognostic features of such a rare tumor entity.

Methods We conducted a retrospective study over a 12 year period (2004–2015) in the Tunisian Central Cancer Registry. We collected all the pathology established cases of ovarian SLCT.

Results The incidence of ovarian SLCT was 1.5% of all the Ovarian, fallopian tube, and peritoneal cancers in our registry. The mean age at the diagnosis was 30 years [14 – 79 ] with a 2-peak distribution: 14 –30 years (46.15% of the patients) and 50 – 80 years. Endocrine symptoms were present in 76,92% of the patients (virilization: 38.46%). Testosterone serum levels were high in 33.33% of the patients. The pathological FIGO staging was IA in 15.38%, IC in 61.53%, and IIIC in 23.07%. A fertility-sparing surgery was performed in 46.15%. Adjuvant chemotherapy (bleomycin, etoposide, and Cisplatin) was delivered in 46.15%. The recurrence rate in the conservatively operated group was 16.67% and the overall progression rate was 47.46%. The overall survival at 5 years was 38%.

Conclusions Our results suggest that ovarian SLCT are rare but can occur at any age, even in menopausal women. The clinical features are essentially endocrine symptoms. In young women, fertility-sparing surgery is feasible but with a 16.67% recurrency rate and 38% 5- year overall survival.