Objective To investigate the diagnosis and treatment of anaplastic carcinoma of the pancreas. Methods The clinical data of 10 patients with anaplastic carcinoma of the pancreas who were admitted to the Zhongshan Hospital from January 1999 to June 2010 were retrospectively analyzed. Computed tomography was carried out preoperatively and surgical plan was designed according to the site of tumors. Chemoradiotherapy was applied postoperatively. Patients were followed up till March 2012 by phone call and outpatient examination. The clinical and imaging features, pathological characteristics, treatment and followup data were analyzed. The clinical and pathological features were analyzed by descriptive statistics. The continuous data were described as ±s, and categorical data were presented by frequency and precentage. Results The tumors located at the head of the pancreas were observed in 5 patients, tumor located at the neck of the pancreas in 1 patient, and tumors located at the body and tail of the pancreas in 4 patients. Two patients underwent pancreaticoduodenectomy (PD), 2 underwent PD+extended lymph node dissection, 1 underwent PD+portal vein reconstruction, 1 underwent pancreatectomy, 4 underwent resection of body and tail of pancreas and splenectomy. The size of the tumors ranged from 2.0 cm×2.0 cm×2.0 cm to 14.0 cm×12.0 cm×9.0 cm. Duodenal and biliary invasion was observed in 4 patients, superior mesenteric veinportal vein invasion in 1 patient, splenic artery invasion in 1 patient.Neural invasion was observed in 8 patients, including 4 patients with lymph node metastasis. The results of immunohistochemical staining showed that 10 patients were with positive expression of cytokeratin 7, and 1 patient was with positive expression of vimentin. Nine patients were followed up, 2 patients did not receive postoperative chemoradiotherapy, 6 received chemotherapy with Gemicitabine and 1 received interventional treatment. The survival time of the 9 patients ranged from 8 to 20 months, and the median survival time was 12 months. Eight patients died of tumor recurrence and metastasis. Conclusions Pancreatic anaplastic carcinoma is a distinct rare variant of pancreatic ductal adenocarcinoma. Pancreatic anaplastic carcinoma has high malignancy, definite diagnosis depends on pathological examination, and the surgical plan should be made according to the result of imaging examination. Conventional radiotherapy and chemotherapy are ineffective for the treatment of this disease.