One of the common malignancies of the gallbladder called Squamous cell carcinoma (SCC). Primary SCC of the gallbladder accounts for about 3.3% of the gallbladder cancers. Pure SCC is a very rare condition with an unknown histogenesis. Herein, we presented a 45-year-old female with a history of vague abdominal pains diagnosed with pure SCC. In the results of histopathological examination a well-differentiated keratinized SCC has been revealed. The patient was subjected to surgery based on the presumptive diagnosis of acute cholecystitis. Considering the poor prognosis of this lesion, the achievement of knowledge on the clinical presentations of this lesion is of paramount importance. The choice of therapeutic strategy for the management of this carcinoma depends on its locoregional extension; however, surgical resection is usually the choice.
