The aims of reporting this case are to describe a rare presentation of AOSD in a middle-age woman with quotidian fever, severe sore throat, and transient skin rashes which initially mimicked an infectious process for over two weeks before admission, and was associated with very high level of immunoglobulin (Ig)-E (also known as hyperimmunoglobulin-E), which seems to be an unusual feature of the disease. Here, IGHE is linked to adult-onset Still disease.