Primary hepatic sarcomas are one of the extremely rare malignant tumors representing less than 0.1 % of all primary malignant neoplasms. A known compensated cirrhotic HCV 60-year-old female lady MELD score 8 with a medical history of controlled diabetes and hypertension presented to our hepatobiliary clinic experiencing a chronic right hypochondrial pain that radiate to her right shoulder of 3 months duration. O/E there was right hypochondrial and epigastric tenderness. Pelvi-abdominal US was done then PET CT which revealed a large exophytic focal lesion measuring about 8 × 7 × 6 cm and achieving 12.4 SUV max on FDG uptake. Pre-operative US guided true-cut biopsy showed small oval rounded cells with focal spindling and the neoplastic cells showed hyperchromatic pleomorphic nuclei with little cytoplasm with a positive reaction of tumor cells stained with Desmin and Myogenin with a diagnosis of pleomorphic rhabdomyosarcoma. The patient was scheduled for neoadjuvant chemotherapy and then elective Right hemihepatectomy. Although many challenges were encountered during the resection of the tumor as the tumor was attached to the under surface of the diaphragm, the tumor was resected with a safety margin of 1 cm with frozen histopathological examination being negative for any malignant cells. The patient's postoperative course was uneventful apart from wound infection and was discharged on the postoperative sixth day. CONCLUSIONS The percutaneous liver biopsy plays an important role in the diagnosis of liver's rhabdomyosarcomas. Neoadjuvant chemotherapy addresses the behavior of the tumor, together with early surgical intervention can lead to favorable outcomes and reduce the recurrence.