Introduction: Lemierre’s syndrome (LS), infectious thrombophlebitis of the internal jugular vein, is a rare, life-threatening complication of oropharyngeal infections underrepresented in literature. We reviewed the etiology, clinical characteristics, treatment regiments and prognosis of LS in pediatric patients.Methods: PubMed and MEDLINE were searched from February 10, 2018 to July 28, 2018 for relevant studies. A systematic review was performed using the terms LS and pediatric case. Our criteria included reviews, case reports, and case series with patients under 18 years with a diagnosis of LS. Results: 70 cases of pediatric LS were identified. The male to female ratio was 1:1.7 with an average age of 10.7 years (5 weeks to 17 years). The most common initial clinical presentations were fever (90.0%), sore throat (38.6%), and neck pain or tenderness (35.7%), while the most frequent findings on physical exam were fever (31.4%) and neck involvement (28.6%). F. necrophorum was the predominant organism cultured (62.9%). Septic emboli were seen in 51.4% of cases with the lungs affected in 40 patients. Most commonly used treatments were antibiotics (91.4%) followed by anticoagulation (50.0%) and the overall mortality rate was 8.6%. The average time from initial presentation to diagnosis was 4.9 days. Conclusion: LS is a deadly condition with a staggering mortality rate despite the advent of antibiotic measures. Early identification and a high index of suspicion are necessary to prevent complications associated with LS. Despite its rarity it should remain on the differential for any patient with toxic signs and localized neck findings.