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Objectives/Hypothesis: Lemierre's syndrome is characterized by a history of recent oropharyngeal infection, clinical or radiological evidence of internal jugular vein thrombosis, and isolation of anaerobic pathogens, mainly Fusobacterium necrophorum. It was once called the forgotten disease because of its rarity, but it may not be that uncommon after all. This review aims to provide physicians with an update on the etiology, management, and prognosis of Lemierre's syndrome. Methods: Systematic review using the terms: Lemierre's syndrome, postanginal septicemia, fusobacterium, internal jugular vein thrombosis. Inclusion criteria: English literature; reviews, case reports, and case series. Exclusion criteria: variants or atypical Lemierre's syndrome cases, negative fusobacteria cultures, and papers without radiological evidence of thrombophlebitis. Results: Eighty-four studies fulfilled our inclusion criteria. The male to female ratio was 1:1, 2, and the ages ranged from 2 months to 78 years (median, 22 years). Main sources of infection were tonsil, pharynx, and chest. Most common first clinical presentation was a sore throat, followed by a neck mass and neck pain. The most common offending micro-organism was F. necrophorum. Treatment modalities used were antimicrobial, anticoagulant, and surgical treatment. Morbidity was significant with prolonged hospitalization in the majority of patients. The overall mortality rate was 5%. Conclusions: Lemierre's syndrome may not be as rare as previously thought. This apparent increase in the incidence may be due to antibiotic resistance or changes in antibiotic prescription patterns. Successful management rests on the awareness of the condition, a high index of suspicion, and a multidisciplinary team approach. Laryngoscope, 2009 [ABSTRACT FROM AUTHOR]