Aortic dissection is the most devastating sequela of thoracic aortic disorder. Patients with acute aortic dissection typically manifest as an acute onset of severe chest pain, but occasionally present with atypical symptoms including fever of unknown origin. A total of 50 patients from 41 articles based on a complete literature retrieval were included in this study. More patients had a fever prior to pain. The time to presentation was 40.7 ± 105.6 days, the time to diagnosis was 52.9 ± 110.1 days, and the time to surgery/intervention was 1.8 ± 5.6 days. The patients' temperature on admission was 38.2 ± 0.6 °C and the maximal temperature recorded was 38.8 ± 0.4 °C. Laboratory findings showed increased white blood cell counts, cardiac enzymes, and inflammatory biomarkers. More pronounced laboratory findings of the infectious type than the inflammatory type aortic dissection could be helpful in the differential diagnosis. Half of patients warrant aortic repair with or without valve replacement, less than half of patients were conservatively managed, and a few were interventionally treated or were being followed up. The mortality rate was 9.5 %. Physicians should always bear in mind aortic dissection when patients present with fever of unknown origin particularly in those without chest pain. Laboratory findings may offer inflammatory evidence for the diagnosis. An early diagnosis as well as subsequent treatment is indispensable for patients' outcomes.