Even with the recent advance in diagnostic tools and techniques, fever of unknown origin (FUO) remains a clinical challenge. A wide range of diseases, mainly infections, autoimmune conditions (inflammatory diseases), malignancies and miscellaneous can cause FUO. Positron emission tomography (PET) or positron emission tomography/computed tomography (PET/CT) scanning makes a great contribution to the diagnosis and differential diagnosis of FUO due to the high sensitivity of pathological accumulation of 18F-FDG. The diagnostic yield of PET/CT is higher than traditional radiographic imaging and other nuclear medicine scanning. Owing to the numerous advantages of PET/CT including high sensitivity and the ability to perform whole-body scans, many rare diseases presenting with FUO can be detected and the spectrum of diseases that can exhibit FUO has been increasing. Recent studies utilizing FUO are discussed in this paper. However, there are limited data available about the role of 18F-FDG PET or PET/CT in evaluation of FUO.