Background: Cystic dystrophy in heterotopic pancreas (CDHP) is a rare condition. It has been recently reported as one of the etiologic obstructive factors of chronic pancreatitis. The aim of our study was to evaluate diagnosis and management of CDHP in the duodenal wall in a surgical series. Methods: We retrospectively reviewed 13 patients with available clinical data. Results: There were 11 male and 2 female patients, median age 42 years. The average duration of symptoms was 7.5 months. 6 of them (46%) were alcoholics, and 10 (75%) had signs of chronic pancreatitis. Almost all of them (12/13; 92%) revealed disabling pain, while 4 (31%) had associated jaundice. In 4 of 7 patients (57%), weight loss was observed. Most often the patients were suspected of having pancreatic head mass with or without signs of chronic pancreatitis. All patients underwent surgical treatment. Pathological examination showed the presence of cysts surrounded by inflammation and fibrosis in the duodenal wall, as well as the presence of chronic pancreatitis in the pancreas proper. Conclusions: Cystic dystrophy of the duodenal wall represents a significant proportion of patients undergoing surgery for chronic pancreatitis. Pancreatoduodenectomy is the best therapeutic option.