Sex cord-stromal tumors (SCSTs) of the ovary are uncommon. Their behavior is unpredictable, often with late recurrence, making counseling, management, and prediction of prognosis challenging. A 65-year-old woman presenting with a SCST underwent a bilateral oophorectomy, the histology was unusual but likely to be a luteinized thecoma with suspicious features for invasion. Two years later following a gastrointestinal bleed, a metastasis within the small bowel mucosa was detected. This represents probable isolated hematogenous or lymphatic spread, which is highly unusual especially in the absence of concurrent peritoneal disease. This is the first reported case of a SCST recurring in small bowel mucosa, mimicking a primary colorectal tumor. This highlights the diverse nature and behavior of these tumors.
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