Lemierre's syndrome is a rare septic thrombophlebitis of the internal jugular vein with frequent metastatic complications following an acute oropharyngeal infection. Immunocompromised patients are at higher risk of developing this syndrome owing to the decreased host response and increased risk of oropharyngeal sepsis. We herein report a 24-year-old woman with severe lupus nephritis on immunosuppressive therapy, who developed Lemierre's syndrome following a pharyngeal infection despite an adequate and timely antibiotic therapy. We hereby draw an attention to the importance of accurate and timely diagnosis and appropriate management of Lemierre's syndrome in immunocompromised patients so as to achieve a successful prognosis for this deadly infection.