Primary gallbladder pleomorphic sarcoma is a very rarely seen type of tumor and its prognosis is not well known mainly due to the limited number of cases reported. Here, we present a case with primary gallbladder pleomorphic sarcoma of which diagnosis was based on morphology and immunohistochemistry. A 70-year-old male presented with three months of abdominal pain in the upper right quadrant and weight loss. The patient reported a past medical history of chronic cholecystitis. Using abdominal magnetic resonance imaging (MRI), the patient was diagnosed with gallbladder cancer. Radical cholecystectomy, with segment 4b and 5 resection of the underlying liver tissue and dissection of the pericholedochal lymph nodes were performed. Patient's postoperative course was unremarkable and he was discharged on the postoperative 5 th day. The immunohistochemical analyses supported the diagnosis of primary gallbladder pleomorphic sarcoma. The patient was referred to the department medical oncology for adjuvant chemotherapy Primary gallbladder pleomorphic sarcoma is a very rare histological type with an unclear pathogenesis. A better understanding of pathogenesis and treatment for this disease, with the larger case series may be better addressed.