Tuberculosis (TB) is one of the major public health threats, competing with the human immunodeficiency virus (HIV) as the cause of death due to infectious diseases worldwide. Extrapulmonary tuberculosis (EPTB) contributes to the burden of disease and does not receive specific attention in international control strategies. We aimed to give an update on the current epidemiological data of EPTB, to describe its clinical spectrum, to identify the main risk factors and to propose effective preventive strategies to reduce EPTB burden. Recent epidemiological data showed that the proportion of EPTB are increasing in different countries, accounting for 15% of the 6.3 million incident cases of TB that were notified in 2016 worldwide. The commonest EPTB reported sites were lymph node, pleura and urogenital tuberculosis. EPTB site has been associated with the immune status of TB patients. The main reported risk factors of EPTB were HIV infection and low CD4 count, female gender, extremes of age, poverty and several host genes. The main way to stop the transmission of this disease is implementation of effective preventive strategies. They consist of early diagnosis and treatment of active TB, the prevention of active disease in latently infected individual's, vaccination and ensure continued surveillance for infected patients as well as active controlling of zoonotic TB. The key to achieving the STOP TB target of global TB eradication by 2050 will be sustained commitment from donors, authorities, effective national TB programs as well as community engagement.