To introduce the early clinical features and the main points of diagnosis and treatment of children with pancreatic pleural fistula presenting massive bloody pleural effusion. The clinical data of symptoms, signs, laboratory examination, diagnosis and treatment methods of pancreatic pleural fistula in a child are presented. A 4-year-old boy presented with one month history of intermittent fever, cough, chest tightness and chest pain. His pleural effusion collected from three thoracic closed drainage was hemorrhagic. He had no positive abdominal signs. His abdominal B-mode ultrasonography showed rough pancreatic echo. The levels of amylase in blood and pleural fluid were 495 U/L and 35 938 U/L, respectively. There was a fistulous tract from the thoracic cavity leading to the pancreas at thoracic surgical exploration. The child was cured by total parenteral nutrition, intravenous infusion of somatostatin and a stent placed in the pancreatic duct by endoscopic retrograde cholangiopancreatography (ERCP). The child with pancreatic pleural fistula in early stage mainly present respiratory symptoms and lack abdominal symptoms, so the diagnosis and treatment is often delayed. Significant increase of pancreatic amylase level in pleural effusion can be an important clue and basis for the diagnosis. If conservative therapy fails, the examination and treatment with ERCP should be considered.