| Summary: | Background: Extra-pulmonary features sometimes occur in association with atypical bacterial pneumonia and include neurologic manifestations, diarrhea, rashes, altered liver enzymes, or kidney injury, among other conditions. Acute pancreatitis has been associated with atypical pneumonias since 1973. Methods: We performed a systematic review of the literature in the Excerpta Medica, National Library of Medicine, and Web of Science databases. We retained 27 reports published between 1973 and 2022 describing subjects with an otherwise unexplained pancreatitis temporally associated with an atypical pneumonia. Results: The reports included 33 subjects (19 males, and 14 females; 8 children and 25 adults) with acute pancreatitis temporally associated with atypical pneumonia caused by <i>Mycoplasma pneumoniae</i> (<i>n</i> = 18), <i>Legionella</i> species (<i>n</i> = 14), or <i>Coxiella burnetii</i> (<i>n</i> = 1). Approximately 90% of patients (<i>n</i> = 29) concurrently presented with respiratory and pancreatic diseases. No cases associated with <i>Chlamydophila pneumoniae</i>, <i>Chlamydophila psittaci</i>, or <i>Francisella</i> species were found. Conclusions: Acute pancreatitis has been associated with various infectious agents. The present review documents the association with atypical pneumonia induced by <i>Mycoplasma pneumoniae</i>, <i>Legionella</i> species, and <i>Coxiella burnetii</i>.
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