Tehran University of Medical Sciences (TUMS) Academic Journal of Surgery 2423-3218 8 3 2025 12 22 106 108 Hüseyin Yönder Private Megapark Hospital, General Surgery Clinic, Kahramanmaraş, Turkey Mehmet Yılmaz Dicle University, Faculty of Medicine, Department of General Surgery, Diyarbakır, Turkey 2025 11 20 Pleomorphic leiomyosarcoma (PLMS) is a rare malignant tumor of smooth muscle origin. Primary hepatic localization is extremely uncommon, with only a few cases reported in the literature. We present a case of primary hepatic PLMS diagnosed and surgically treated in our institution. An 83-year-old female presented with abdominal pain and vomiting. Her medical history included previous surgery for hepatic hydatid cyst. Laboratory analysis revealed elevated cholestatic enzymes and bilirubin levels. Dynamic liver CT showed a 200×136 mm hypodense lesion with septal enhancement in the right lobe extending partially to the left (Figure 1). Thoracic CT demonstrated right pleural effusion. After preoperative optimization, surgery was performed. Intraoperatively, a 30×20 cm semisolid mass was identified, and frozen section analysis suggested a borderline tumor. Extended right hepatectomy (trisectionectomy) involving the right and middle hepatic veins was performed. The postoperative course was uneventful, and the patient was discharged. Final histopathological examination confirmed the diagnosis of pleomorphic leiomyosarcoma. Primary hepatic PLMS is exceedingly rare. Curative surgical resection remains the mainstay of treatment and offers the best chance for long-term survival. This case highlights the importance of aggressive surgical management in rare hepatic sarcomas. https://ajs.tums.ac.ir/index.php/ajs/article/view/333