Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumor. The tumor has no specific presentations and often diagnosis is delayed until it reaches a significant size. We report the case of a 69-year-old female presented with a huge PHL. Due to size of the tumor and to be operable, the patient subjected to right portal vein embolization (PVE) and selective embolization of segment V. Four weeks after the PVE, liver resection was conducted (Segments V+VI bisegmentectomy plus resection of IVA). The patient had an uncomplicated post-operative course, and discharged at the 8th post-operative day. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. The patient refused to receive adjuvant chemotherapy, and revealed evidence of recurrence six months after the operation, and finally died 12 months after the operation and 16 months after initial diagnosis. PHL is an extremely rare tumor and often in first presentation has significant size. Radical surgery with adjuvant chemotherapy is key feature for prolonged survival.