Abstract: 　Objective　To explore the clinical characteristics, diagnosis and treatment of massive hemorrhagic pleural effusion caused by pancreaticopleural fistula (PPF) in children. Method　The diagnosis and treatment of a child with massive right-sided hemorrhagic pleural effusion caused by PPF was retrospectively analyzed. The Chinese databases and PubMed databases were searched up to August 2018 for literature review. Results　An 8-year-old girl suffered from intermittent dyspnea for more than 20 days. CT showed massive pleural effusion at the right side and signs of chronic pancreatitis. Although symptomatic treatment was given, bloody fluid continued to flow out from the closed chest drainage tube, and the highest amylase in pleural effusion was 56365.7 U/L. PPF was found by second imaging examination. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic retrograde pancreatic drainage were performed and the girl was improved after treatment and discharged. Literature review identified 18 cases of PPF in children from 6 Chinese articles and 6 English articles and main manifestations were dyspnea or chest tightness (13 cases) and abdominal pain (10 cases). Except for 2 patient who was not clearly reported, amylase/lipase in pleural effusion was increased in other patients who were confirmed by CT, magnetic resonance cholangiopancreatography, ERCP and surgical exploration respectively. Surgery, ERCP intervention or conservative treatments were effective. Conclusions　PPF is a rare pancreatic complication in children and has significant pulmonary symptoms. Pleural effusion amylase detection should be carried out as soon as possible, and fistula should be actively sought. After treatment, the prognosis is mostly good.

Key words: pancreaticopleural fistula;　pleural effusion;　child