Although a mucoepidermoid carcinoma is sometimes seen as a neoplasm originating from the salivary glands, its origination from the liver is rare. A review of the medical literature in the English language disclosed only 16 cases originating from the hepatic system. The case explored here is that of an 81-year-old female, without any significant medical history, who was referred to our hospital for elevated liver function tests. Computed tomography (CT) showed a huge tumor, 10 cm in diameter, in the right lobe of the liver. The central part of the tumor was consistently un-enhanced, suggesting a large necrotic area. The liver tumor was diagnosed as mucoepidermoid carcinoma by a percutaneous biopsy. Despite chemotherapy with radiation therapy, the tumor showed very aggressive malignancy, resulting in early mortality. We herein report on a rare case of primary mucoepidermoid carcinoma of the liver with pulmonary and lymph node metastasis, confirmed by autopsy, and review the literature.