Acinar cystic transformation (ACT) is a rare cystic lesion of the pancreas lately reclassified as a non-neoplastic entity. This is a presentation of a case of ACT and a review of the literature. A systematic PubMed search was conducted, yielding a result of 24 publications. Including our case report, 75 cases of ACT have been reported in the literature. The patients are mainly females with initial symptoms of abdominal pain. The cysts are primarily located in the head of the pancreas and are often multilocular on cross section and have a mean size of 53.2 mm. Microscopically, the cysts are lined by an acinar epithelium with abortive acinar formations. The cells are immunohistochemically positive in stains for trypsin, chymotrypsin and CK7. The Ki67-index is low; 1-2 %. No genetic alterations indicative of a neoplastic pathogenesis have been found. The mean follow up time is 37.4 months and no malignant transformation has been observed. A preoperative diagnosis is difficult to establish, but microbiopsies seem a promising tool. The indication for surgical intervention should be founded on the symptoms of the patients since no malignant transformation has ever been reported.