Acute acalculous cholecystitis (AAC) is an uncommon presentation of cholecystitis accounting for 10% of cases. AAC is caused by a localized ischemic event in the gallbladder usually in critically ill patients. Several cases of localized or systemic lymphoma have been described in the literature as causes of AAC. We present a patient with a rare case of AAC due to undiagnosed systemic Burkitt lymphoma. Pathology of the gallbladder revealed Burkitt lymphoma with analysis of his cerebral spinal fluid confirming Stage IV disease. This case report reviews acute acalculous cholecystitis, lymphomas of the gallbladder and extrahepatic duct, and adult Burkitt lymphoma.