Objective: Mycoplasma pneumoniae is responsible for approximately 20% of community-acquired pneumonia. A wide variety of extrapulmonary manifestations related to M. pneumoniae infection can occur. The diagnosis of M. pneumoniae and its association with acute pancreatitis is briefly reviewed. Clinical Presentation: We describe the case of a 9-year-old boy with clinical features of acute abdomen. Intervention: A perforated appendix was suspected and the patient was accordingly operated on. The clinical, radiological and serological findings that led to the proper diagnosis are described. Conclusion: The case is presented to help increasing the awareness of M. pneumoniae infection as a potential cause of acute pancreatitis in children.