Extreme and compound events disrupt lake ecosystems worldwide, with their frequency, intensity and duration increasing in response to climate change. In this Review we outline evidence of the occurrence, drivers and impact of extreme and compound events in lakes. Univariate extremes, which include lake heatwaves, droughts and floods, underwater dimming episodes and hypoxia, can occur concurrently, sequentially or simultaneously at different locations to form multivariate, temporal or spatial compound events, respectively. The probability of extreme and compound events is increasing owing to climate warming, declining lake water levels in half of lakes globally, and basin-scale anthropogenic stressors, such as nutrient pollution. Most in-lake extreme events are inherently compound in nature owing to tightly coupled physical, chemical and biological underlying processes. The cascading effects of compound events propagate or dissipate through lakes. For example, a heatwave might trigger stratification and oxygen depletion, subsequently leading to fish mortality or the proliferation of harmful algal blooms. Interactions between extremes are increasingly observed and can trigger feedback loops that exacerbate harmful algal blooms and fishery declines, leading to severe ecological and socio-economic consequences. Managing the increasing risk of compound events requires integrated models, coordinated monitoring and proactive adaptation strategies tailored to the vulnerabilities of lake ecosystems.