We report the first imported case of chronic Q fever with multi-organ involvement seen in Hong Kong. Although the disease is found worldwide, its chronic form is very rare in our locality. Familiarity with the clinical presentation, useful diagnostic tools, and appropriate treatment is necessary for the prevention of the serious morbidity and mortality associated with chronic Q fever. To the best of our knowledge, this article represents the first comprehensive review to compare the local experience with Q fever with international data, and establishes a management approach for this unusual infectious disease while suggesting possible explanations for its exceptionally low incidence in this locality.