Extrapulmonary tuberculosis (EPTB) is defined as TB disease outside the lung parenchyma and can involve any organ in the body. EPTB represents about 30% of all TB, although the incidence varies across settings. The most common localization of EPTB is in the lymph nodes, primarily cervical lymph nodes, and is a common manifestation in pediatric TB. Hematogenous spread of M. tuberculosis may cause localized disease or miliary TB with multiple small granulomas seeded throughout the body. About one-third of all patients with miliary TB have central nervous system (CNS) involvement. TB in the CNS, such as TB meningitis, have a high mortality and morbidity, and rapid diagnosis is vital. In this chapter, we will review the clinical characteristics and diagnostic procedures for various manifestations of EPTB such as lymph node, skeletal, abdominal, and urogenital TB and with special reference to difficult-to-diagnose conditions such as TB pericarditis and CNS TB.