The majority of pancreatic malignant tumours are adenocarcinomas of the ductal type, whereas acinar cell carcinoma (ACC) is unusual. Its management is not well codified, resulting from its low frequency. It seems however that curative operative resection represents the best first-line treatment. We present a case of ACC in a 54-year-old woman who presented with right upper quadrant pain of the abdomen due to a pancreatic mass in the head of the pancreas, detected by computer tomography (CT) and magnetic resonance imaging (MRI). In April 2008 the patient underwent a pancreatoduodenectomy (Longmire-Traverso) and received postoperative chemotherapy. We present a case of pancreatic ACC treated with a curative operation followed by chemotherapy. Our patient remains free of the disease and has a good quality of life.