BACKGROUND: Primary leiomyoma of the ovary is a rare benign ovarian tumor that only seldom causes acute abdomen.CASE SUMMARY: A 35-year-old gravida 1, para 0 woman presented with a history of acute lower abdominal pain, and 10 weeks of amenorrhea. The patient's physical examination revealed abdominal tenderness, defense, and rebound. On ultrasonographic examination, a solid mass measuring 9.3 * 7.8 cm was detected adjacent to the uterine fundus. The mass was preoperatively diagnosed as a twisted pedunculated subserosal uterine myoma. Upon entering the pelvic cavity, the mass in the right adnexa appeared twisted clockwise. Therefore, a laparoscopic salpingo-oophorectomy was performed. The tumor was pathologically diagnosed as ovarian leiomyoma. The patient delivered a healthy girl at 40 1/7 weeks of pregnancy.CONCLUSION: Despite its low incidence, torsion of ovarian leiomyoma should be considered in the differential diagnosis of acute abdomen. Furthermore, laparoscopic exploration should be the preferred way of removing twisted ovarian leiomyoma, even during pregnancy. It seems that primary ovarian leiomyomata have a tendency to grow rapidly during early pregnancy. However, because of the low incidence of ovarian leiomyoma, the effects of estrogen and pregnancy on this condition remain unclear.