A 7-year-old male patient (the proband, IV-4;  Figure 1A  ) had a history of two episodes of mycobacterial lymphadenitis and recurrent typhoid fever. He was referred to the pediatric immunology clinic due to a newly diagnosed disseminated tuberculosis (TB) affecting the lungs and multiple lymph nodes that included the mesenteric lymph nodes. The patient had a history of fever and left axillary lymphadenitis at the age of 6 months, for which he received oral and injectable antibiotics without improvement. Excisional biopsy and histopathology showed caseating granuloma diagnosed as BCGitis, and he was treated with four anti-TB drugs (rifampicin, isoniazid, pyrazinamide, and ethambutol) for only 6 months. At 2 years of age, he developed fever, weight loss, and cervical lymphadenopathy. Fine-needle aspiration cytology showed caseating granuloma. He received the same combination of anti-TB drugs as before for 6 months, and his symptoms improved (  Figure 1B  ).