Background: Fusobacterium species are anaerobic Gram-negative bacilli that are part of the normal oropharyngeal and gastrointestinal flora. Although rare, they can cause infective endocarditis (IE), a severe condition with high morbidity. The clinical characteristics, treatment strategies, and outcomes of IE caused by Fusobacterium spp. remain incompletely defined. This systematic review aimed to synthesize available data on Fusobacterium IE and compare its features with IE caused by other pathogens. Methods: We conducted a systematic literature search in PubMed, Scopus, and the Cochrane Library up to 27 February 2025, using the terms “Fusobacterium” and “endocarditis.” Eligible studies included case reports or series describing adult or pediatric patients with Fusobacterium-associated IE. Data were extracted on demographics, risk factors, clinical features, microbiology, treatment, and outcomes. Results: A total of 21 studies (all case reports) involving 21 patients were included. The median age was 48 years, and 85.7% were male. Poor dental hygiene or recent dental work was the most common predisposing factor (47.6%). The mitral valve was most frequently affected (44.4%). Fever and sepsis were reported in nearly all cases, and embolic phenomena occurred in 81%. The most commonly isolated species were F. necrophorum (47.6%) and F. nucleatum (42.9%). Treatment commonly included metronidazole (61.9%), while surgical management was required in 23.8%. All-cause and infection-attributable mortality were both 9.5%. Conclusions: Fusobacterium IE predominantly affects younger males and is often linked to oral sources. This disease is associated with a high risk of systemic complications but seems to have a lower mortality compared to IE from other pathogens, including other anaerobic bacteria. Early diagnosis and appropriate antimicrobial treatment are of utmost importance for optimal outcomes. Further research is required to guide evidence-based management of this rare but serious infection.