Background: Uterine tumor similar to ovarian sex cord tumor (UTROSCT) is a rare disease, with few cases reported in the literature and uncertainty regarding its origin and characteristics. Case Description: The patient described is a 48-year-old woman who presented to the gynecological clinic with persistent metrorrhagia and pelvic pain. On ultrasound examination, an increase in uterine volume and a mass measuring 3.22 cm x 2.54 cm similar to a uterine fibroid were observed. Six months after the examination, the patient repeated the ultrasound which showed the appearance of 2 more leiomyomatous lesions measuring 50.99 mm x 46.72 mm and 26.48 mm x 20.21 mm respectively, leading to the indication of total hysterectomy and bilateral salpingectomy. During surgery, she began laparoscopy and, after a 2-cm injury to the urinary bladder associated with several peritoneal adhesions, she underwent laparotomy surgery. The differential diagnosis of other diseases that affect the uterus was made exclusively through postoperative histopathological examination, confirming the diagnosis of UTROSCT. The tumor appears, macroscopically, as a single, yellowish, and well-defined uterine fibroid measuring 50 mm in the largest axis and located intramurally. The tumor's histology shows lesions described by infiltration with a diffuse, chordal and microtubule arrangement, composed of small to medium-sized cells with ovoid nuclei, a histological pattern similar to ovarian sex cord tumors. The definitive diagnosis was made in the immunohistochemical analysis which showed: estrogen receptor (+), CD10 (+), WT1(+), calretinin (+), AE1/AE3 (+), 1A4 (+), FOXL2 (-), inhibin (-), desmin (-), melan-A (-) EMA (-). The patient's condition was benign and without recurrence so far, demonstrating that the surgery was sufficient to cure the patient and that the use of chemotherapy treatments was not necessary. Conclusions: This case corroborates the projected results that a surgical intervention can achieve, preventing metastases and complications.