Abstract:Background and Aims Acute pancreatitis (AP) is an acute inflammatory disease of the pancreas caused by abnormal activation of pancreatic enzymes. Although gallstones, hyperlipidemia, and alcohol use are the most common causes, a subset of patients develop AP secondary to rare etiologies that are often misdiagnosed or diagnosed late, leading to recurrence or inappropriate management. This study aims to summarize the clinical characteristics, diagnostic strategies, and treatment outcomes of four cases of AP caused by uncommon etiologies, supported by a literature review.Methods Clinical data of 4 patients admitted to the Department of Hepatobiliary and Pancreatic Surgery, the Third Xiangya Hospital of Central South University, between November 2021 and September 2024, were retrospectively analyzed. Their etiological characteristics, diagnostic approaches, and treatment strategies were reviewed in combination with relevant literature.Results The underlying causes of AP were intraductal papillary mucinous neoplasm, pancreatic neuroendocrine tumor, pancreatic ductal adenocarcinoma, and duodenojejunal intussusception. All cases initially presented as idiopathic AP. Three patients underwent definitive surgical treatment and recovered well, while one patient with pancreatic cancer received only palliative care due to delayed diagnosis and died three months later.Conclusion AP secondary to rare etiologies often mimics common forms in clinical presentation but poses diagnostic challenges. For patients with recurrent or idiopathic AP, clinicians should emphasize etiological tracing and utilize advanced imaging and endoscopic modalities for early identification. Timely etiological intervention, particularly surgical management when appropriate, is essential for preventing recurrence and improving prognosis.