In 1977, a case report was published describing a 28-year-old women with an endometrial stromal tumor that showed foci of myogenic differentiation. The term "stromomyoma" was introduced to encompass both this type of neoplasm as well as "uterine neoplasms resembling ovarian sex-cord tumors" (UTROSCTs). More than 17 years later, the tumor recurred, involving the right ovary, sigmoid colon, small bowel, abdominal wall and omentum. The histologic and electron microscopic similarities between the recurrent tumor and the primary neoplasm were confirmed. Applying the recent classification and diagnostic criteria of endometrial mesenchymal neoplasms, we have concluded that this tumor was a low-grade endometrial stromal sarcoma (LGSS). The formerly proposed term "stromomyoma" implies a benign tumor, in contrast to the obviously malignant nature of this particular tumor. Focal myogenic differentiation of LGSS is not an uncommon finding and does not warrant a separate diagnostic or prognostic entity. UTROSCTs and endometrial stromal sarcomas are two separate diagnostic entities, and combining them under an inclusive terminology is not appropriate.