With rapid urbanization, population growth and anthropogenic activities, an increasing number of major cities across the globe are facing severe urban heat islands (UHI). UHI can cause complex impacts on the urban environment and human health, and it may bring more severe effects under heatwave (HW) conditions. In this paper, a holistic review is conducted to articulate the findings of the synergies between UHI and HW and corresponding mitigation measures proposed by the research community. It is worth pointing out that most studies show that urban areas are more vulnerable than rural areas during HWs, but the opposite is also observed in some studies. Changes in urban energy budget and major drivers are discussed and compared to explain such discrepancies. Recent studies also indicate that increasing albedo, vegetation fraction and irrigation can lower the urban temperature during HWs. Research gaps in this topic necessitate more studies concerning vulnerable cities in developing countries. Moreover, multidisciplinary studies considering factors such as UHI, HW, human comfort, pollution dispersion and the efficacy of mitigation measures should be conducted to provide more accurate and explicit guidance to urban planners and policymakers.