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The burden of childhood tuberculosis (TB) is influenced by the human immunodeficiency virus (HIV) epidemic and this dangerous synergy affects various aspects of both diseases; from pathogenesis and the epidemiologic profile to clinical presentation, diagnosis, treatment, and prevention. HIV-infected infants and children are at increased risk of developing severe forms of TB. The TB diagnosis is complicated by diminished sensitivity and specificity of clinical features and diagnostic tools like the tuberculin skin test and chest x-ray. Although alternative ways of pulmonary sampling and the development of interferon-γ assays have shown to lead to some improvement of TB diagnosis in HIV-infected children, new diagnostic tools are urgently needed. Coadministration of anti-TB treatment and antiretroviral drugs induces severe complications, and this highlights the need to define optimal treatment regimens. Practical implementation of these regimens in TB control programs should be combined with isoniazid preventive therapy in TB-exposed HIV-infected children. The risk of severe complications after Bacille Calmette-Guérin vaccination of HIV-infected children emphasizes the need for new nonviable vaccines. This article reviews the current status of pediatric HIV-TB coinfection with specific emphasis on the diagnosis and treatment.