Abstract:  Objective　To explore and provide guidelines for the clinical diagnosis and treatment of primary nephrotic syndrome complicated with acute pancreatitis. Methods　The clinical data of 14 children with primary nephrotic syndrome complicated with acute pancreatitis during September 2013 to September 2016 were retrospectively analyzed. Results　In 14 children (6 males and 8 females) aged 3 to 15 years. all children presented massive proteinuria, hypoalbuminemia, varying degrees of edema, hyperlipidemia and pain in upper abdomen or left hypochondrium. Seven children had nausea and vomiting, and their amylase in serum and urine fluctuated at 392?802 U/L and 561?3180 U/L, and the lipase level was 339.1±2.52 U/L. After supportive treatment, 13 children were cured from pancreatitis except one who gave up the treatment. Conclusion　Due to infection, coagulation disorder, hyperlipidemia and drug application in primary nephrotic syndrome, acute pancreatitis may be induced. Clinician should be alerted to it and early diagnosis and treatment were needed for acute pancreatitis.