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Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy presumed to result from an infection-triggered autoimmune reaction.This paper describes a 53-year-old man admitted to hospital for deterioration of his general condition.He developed GBS, confirmed by lumbar puncture and electromyogram, which recovered after intravenous immunoglobulins. A grade 2 aortic regurgitation was detected by transthoracic echocardiography upon diagnosis of GBS, but in the absence of fever, no further investigations were conducted. A few weeks later, the patient presented with fever and infective endocarditis (IE) was diagnosed after the identification of vegetation on the aortic valve with transesophageal echocardiography. The etiologic agent was identified as Cardiobacterium hominis based on 3 positive blood cultures and DNA detection in valvular material.IE was cured with a 6-week course of antibiotics and aortic valve replacement.The patient completely recovered from Guillain-Baré syndrome and IE.This case of GBS associated with C hominis endocarditis, emphasizes the importance of blood cultures and transesophageal echocardiography for the detection of IE and highlights the insidious nature of C hominis endocarditis which is often diagnosed late.