Small cell carcinoma of the ovary hypercalcemic type (SCCOHT) is an exceedingly rare aggressive malignancy. It typically affects young women. Herein we aimed to report a case of a 28-year-old woman treated for SCCOHT in Salah Azaiez Institute. The tumor was revealed by a delay of menses. The imaging found a large mass of the right ovary. The patient underwent a bilateral salpingo-oophorectomy, a total hysterectomy and a pelvic and a para-aortic lymphadenectomy. She subsequently received 6 cycles of adjuvant chemotherapy. Three years later, she presented a metastatic bone relapse.