This nationwide population-based cohort study investigated the risk of tuberculosis (TB) in patients with end-stage renal disease (ESRD) and receiving dialysis. The evaluations included 4131 incident ESRD patients receiving dialysis and 16,524 age- and gender-matched controls, recruited between 1998 and 2009. We used Cox proportional hazards regression analysis to measure the association between TB and ESRD. Compared to the controls, the ESRD cohort had a significantly higher risk of TB within 1 year [incident rate ratio (IRR) 4.13], and 1-2 years (IRR 2.12), of occurrence of ESRD. The Cox proportional hazards model revealed that ESRD [hazard ratio (HR) 2.40], age >65 years (HR 2.41), male sex (HR 1.94), diabetes mellitus (HR 1.36), silicosis (HR 7.70) and chronic obstructive pulmonary disease (HR 1.61) are independent risk factors for TB. Patients with ESRD are associated with an increased risk of TB, and should thus be monitored more carefully for TB, especially within 2 years of onset of ESRD.