Background: Leiomyosarcoma (LMS), is a relatively rare malignant tumor of smooth muscle origin that pose diagnostic dilemma with no standard established treatment. It is characterized by an aggressive behavior, with high rates of local recurrence and metastasis, leading to an early death.

Case report: Mrs. C. N. O, was a 55-year-old Primary school teacher who was a P₂⁺¹ A₂ whose LCB was 22 years and her last pregnancy was 12 years prior to presentation. She presented with abdominal swelling of 11 months’ duration following a total abdominal hysterectomy with bilateral salpingo-oophorectomy on account of symptomatic uterine fibroid. There was associated worsening difficulty in breathing. Her respiratory rate was 24 cycles/minute with dullness on the left lower zone. The liver was enlarged and measured about 5 centimeters below the right coastal margin with additional abdominal mass of about 18 weeks’ uterine size. She was managed for metastatic leiomyosarcoma but could not make it and died while being prepared for palliative care.

Conclusion: Leiomyosarcoma uteri is a highly rare condition with high mortality rate when it occur just as in this index patient. There is need for clinicians to be vigilant in evaluating cases of leiomyomas to avoid missing such cases to minimize mortalities.

Keywords: Leiomyosarcoma, Women