Cystic dystrophy of the duodenal wall is a rare form of the disease which was described in 1970 by French authors who reported the presence of focal pancreatic disease localized in an area comprising the C-loop of the duodenum and the head of the pancreas.Ger-man authors have defined this area as a“groove”.We report our recent experience on cystic dystrophy of the paraduodenal space and systematically review the data in the literature regarding the alterations of this space.A MEDLINE search of papers published between 1966 and 2010 was carried out and 59 paperswere considered for the present study;there were 19 cohort studies and 40 case reports.The majority of patients having groove pancreatitis were middle aged.Mean age was significantly higher in patients having groove carcinoma.The diagnosis of cystic dystrophy of the duodenal wall can now be assessed by multi-detector computer tomography,magnetic resonance imaging and endoscopic ultrasonography.These latter two techniques may also add more information on the involvement of the remaining pancreatic gland not involved by the duodenal malformation and they may help in differentiating“groove pancreatitis”from “groove adenocarcinoma”.In conclusion,chronic pan-creatitis involving the entire pancreatic gland was present in half of the patients with cystic dystrophy of the duodenal wall and,in the majority of them,the pan-creatitis had calcifications. Cystic dystrophy of the duodenal wall is a rare form of the disease which was described in 1970 by French authors who reported the presence of focal pancreatic disease localized in an area comprising the C-loop of the duodenum and the head of the pancreas. Ger- man authors have defined this area as a “groove ”. We report our recent experience on cystic dystrophy of the paraduodenal space and systematically review the data in the literature regarding the alterations of this space. A MEDLINE search of papers published between 1966 and 2010 was carried out and 59 paperswere considered for the present study; there were 19 cohort studies and 40 case reports.The majority of patients with groove pancreatitis were middle ages .Mean age was significantly higher in patients with groove carcinoma. Diagnosis of cystic dystrophy of the duodenal wall can now be assessed by multi-detector computer tomography, magnetic resonance imaging and endoscopic ultrasonography. The latter latter two techniques may al so add more information on the involvement of the remaining pancreatic gland not involved by the duodenal malformation and they may help in differentiating “groove pancreatitis ” from “groove adenocarcinoma ”. In conclusion, chronic pan-creatitis involving the entire pancreatic gland was present in half of the patients with cystic dystrophy of the duodenal wall and, in the majority of them, the pan-creatitis had calcifications.