Introduction Lemierre syndrome (LS) involving the external jugular vein (EJV) is rare, and only a few cases have been reported in the literature. Objectives To report a case of LS involving the external jugular vein as well as to make a review of the literature regarding both diagnosis and management strategies. Data Synthesis We describe a case of LS involving the EJV and review the literature of previously published articles to search for additional cases. A PubMed, Embase, Scopus, and Web of science-based search was performed to determine the scope of coverage in well-reported articles in English. Twenty-one papers were retrieved and documented for age, incidence, pathogen, presenting symptoms, imaging, treatment, and outcome, which were noted for each of these cases. In our literature review of 21 papers, there were 16 patients (61%) in their 2nd and 3rd decades of life. Lemierre syndrome was shown to affect females and males equally. The presenting symptoms were a sore throat and fever. Treatment requires intravenous antibiotics, and there is no consensus regarding treatment with anticoagulation. Conclusions The present case report and review of the literature emphasize the importance of history taking as well as physical examination in what seems to be a case of simple tonsillitis.