Anaplastic carcinoma of the pancreas (ACP) is a very rare histologic subtype of pancreatic cancer and associated with more aggressive and poor prognosis than pancreatic ductal adenocarcinoma. We aimed to review this rare entity and discuss its clinical features, diagnosis and therapy. We presented a case of a 63-year-old male patient that diagnosed as ACP with cyst formation at a tertiary medical center with a detailed review of the current medical literature. We performed pancreaticoduodenectomy operation with lymph node dissection after diagnosis. Any complication after surgery was not observed. Anaplastic pancreas carcinomas are associated with poor survival when compared to invasive ductal adenocarcinomas. Clinical, radiological, laboratory and histological features may be helpful in making differential diagnosis and should be kept in mind in the diagnosis of this rare pancreatic malignancy.
