To summarize the experience of diagnosing and treating patients with Henoch-Schönlein purpura (HSP)-related pancreatitis, a systematic review of previously published cases was conducted. Among 13 reported cases, there were six males and seven females whose age from 3 to 70 years. The clinical features of these patients indicated that acute pancreatitis could be the initial manifestation of HSP, the radiological change was atypical, and most cases were alleviated with steroidal treatment. Good outcomes can be achieved in patients who are diagnosed early with HSP-related pancreatitis, and it is vital to begin timely treatment of HSP-related pancreatitis with corticosteroid.