Soft tissue sarcomas constitute a heterogeneous group of neoplasms of various histologies and comprise less than one per cent of all adult malignancies. They occur at all anatomic body sites, and about 10%-20% of soft tissue sarcomas arise from the retroperitoneum. Leiomyosarcoma, a rare malignancy of smooth muscle, represents 10%-37% of all soft tissue sarcomas arising in the retroperitoneum. Retroperitoneal leiomyosarcoma occurs most commonly in the fifth to seventh decades. There has no known predilection for race or gender. The most common clinical picture at presentation in reported cases includes back pain, weight loss, and increase abdominal girth. Complete surgical resection is the treatment of choice. Adjuvant therapy with radiation is commonly recommended for patient with high-grade resected tumors. The benefits of adjuvant chemotherapy remain controversial. Treatment of this rare neoplasm is complicated by the large size of the tumor at diagnosis and frequent presence of metastases; therefore, prognosis is poor. Herein, we present a 68-year-old woman with increasing abdominal girth, intermittent abdominal discomfort, a palpable abdominal mass, and a 6 kg weight loss over 2 months. Computed tomography of the abdomen disclosed a huge retroperitoneal tumor. Leiomyosarcoma was proved after surgery. Then, she has received radiotherapy. She is currently alive 5 months after diagnosis without recurrence or metastasis.