Background: Scleroderma is a chronic, multisystem, autoimmune disease. Previous studies have shown an increased risk of malignancy in scleroderma; the most common cancers were lung cancer and breast cancer. Case: The patient, a 43-year-old nulliparous premenopausal Greek woman with scleroderma, presented with a history of abdominal pain and atypical vaginal bleeding. She underwent total hysterectomy with bilateral salpingo-oophorectomy, total omentectomy, appendectomy and pelvic lymph node dissection. The histopathology revealed synchronous primary cancers of the endometrium and left ovary. She underwent postoperative chemotherapy and remains well without evidence of disease 25 months after surgery. Conclusion: Synchronous primary cancers of the endometrium and ovary are relatively uncommon in the general population. Only a few cases of cancer of the female genital tract in women with scleroderma have been reported in the English literature.