Aim: The aim of this study was to determine whether there is a match between the health problems that children face and the clinical trials that are being conducted, especially in low- and middle-income countries (LMICs). Methods: All trials on the World Health Organisation International Clinical Trials Registry Platform registered from 2005 to 2013 were reviewed. The disease-specific focus of registered trials for children was compared to the global burden of disease for 2011 using World Health Organisation disability-adjusted life-year data. Results: Children account for 34% of the global disease burden, but were represented in only 15% (29 899/203 726) of registered trials. Although the number of trials in children registered annually has increased over time (from 3174 in 2005 to 3392 in 2013), the proportion of trials involving children has decreased (from 18 to 13%). LMICs account for 98% of the disease burden among children world-wide, but only 22% of trials. More trials were registered in high-income countries than in LMICs for the majority of the disease categories. There was moderate correlation between the number of trials and the burden of each disease within each region (Spearman's correlation r = 0.6, P = 0.007 and r = 0.55, P = 0.02 in LMICs and high-income countries, respectively). Conclusion: Overall, children continue to be under-represented in clinical trials, particularly children from LMICs where disease burden is greatest. Clinical trial activity correlates moderately with disease burden among the world's children. A more evidence-informed approach for prioritising trials to specifically address the health-care needs of children is required.