Signet ring stromal cell tumor is a rare, benign ovarian neoplasm thought to arise from ovarian stromal cells. The pathophysiology of these tumors is poorly understood. They present in women in a wide age range, often with nonspecific symptoms including lower abdominal or pelvic pain. Their morphologic appearance raises a critical differential diagnosis of Krukenberg tumor, an aggressive malignancy with significant implications for patient management. For this reason, it is important for the pathologist to be aware of signet ring stromal cell tumor and its differentiating features, including useful histochemical and immunohistochemical ancillary tests. These tumors are curable with surgical excision, and there have been no recurrences or metastases among reported cases.