Genitourinary tuberculosis (GUTB) is the second most common form of extrapulmonary tuberculosis, with more than 90% of cases occurring in developing countries. Postmortem studies--from before anti-TB therapy was available--have provided insight into the prevalence and natural history of the disease. In GUTB, the kidneys are the most common sites of infection and are infected through hematogenous spread of the bacilli, which then spread through the renal and genital tract. Diagnosis of TB is often delayed owing to the nonspecific nature of its presentation; therefore, a high degree of suspicion should be exercised and a systematic approach should be taken during investigation. Appropriate culture samples should be obtained to tailor treatment. Standard treatment should be administered for 6 months; quadruple therapy for 2 months and dual therapy for 4 months. However, additional drugs and prolonged treatment are required if drug resistance occurs. Although the role of surgery in GUTB has decreased since the advent of anti-TB therapy, it can still have a role as an adjunct to drug treatment. Today, the challenges of GUTB and other forms of TB include increasing rates of drug-resistant cases and co-infection with HIV.