Two cases of endometrial low-grade stromal sarcoma with ovarian sex cord-like differentiation occurring in a 39-year-old woman and a 42-year-old woman are presented. Both tumors, which were intramyometrial and measured 7.5 cm and 7.0 cm in greatest diameter, respectively, showed a multinodular, ill-demarcated, and yellowish white cut-surface. Histologically, most parts of the tumors were composed of trabecular, cord-like, or plexiform arrangements that were reminiscent of the growth pattern seen in ovarian sex cord tumors. Features of conventional endometrial low-grade stromal sarcoma were only focally observed. The tumors showed infiltrative margins and lymphatic invasion. The tumor cells were positive for vimentin, desmin, alpha-smooth muscle actin, and muscle actin (HHF35). The tumors were also positive for both estrogen and progesterone receptors. Both tumors were DNA diploid as determined by flow cytometry. One patient had recurrences, including osteolytic lesions in the pelvic bones, but had no evidence of recurrence or metastasis 11 months after the last surgery. The other patient had no evidence of tumor in a limited follow-up. Familiarity with the neoplasm and other uterine mesenchymal tumors with ovarian sex cord-like differentiation by gynecologists and pathologists is essential in avoiding misdiagnosis because adjuvant hormonal therapy may be effective in treating low-grade stromal sarcomas.