Acinar cell carcinoma of the pancreas is a rare tumor for which the best chemotherapy regimen has not been clearly established. Here, we report on a female patient with an unusually long survival and a remarkable response to weekly paclitaxel. To our knowledge, this is the first time that paclitaxel has been associated with an objective response in this disease. The patient recurred after initial resection and failed multiple prior chemotherapeutic regimens. She received weekly paclitaxel at 80 mg/m2 and responded after eight doses, maintaining controlled disease for a total of 4 mo. We present her case in detail and review the available literature regarding this rare type of tumor.