In contrast to C(3) photosynthesis, the response of C(4) photosynthesis to water stress has been less-well studied in spite of the significant contribution of C(4) plants to the global carbon budget and food security. The key feature of C(4) photosynthesis is the operation of a CO(2)-concentrating mechanism in the leaves, which serves to saturate photosynthesis and suppress photorespiration in normal air. This article reviews the current state of understanding about the response of C(4) photosynthesis to water stress, including the interaction with elevated CO(2) concentration. Major gaps in our knowledge in this area are identified and further required research is suggested.Evidence indicates that C(4) photosynthesis is highly sensitive to water stress. With declining leaf water status, CO(2) assimilation rate and stomatal conductance decrease rapidly and photosynthesis goes through three successive phases. The initial, mainly stomatal phase, may or may not be detected as a decline in assimilation rates depending on environmental conditions. This is because the CO(2)-concentrating mechanism is capable of saturating C(4) photosynthesis under relatively low intercellular CO(2) concentrations. In addition, photorespired CO(2) is likely to be refixed before escaping the bundle sheath. This is followed by a mixed stomatal and non-stomatal phase and, finally, a mainly non-stomatal phase. The main non-stomatal factors include reduced activity of photosynthetic enzymes; inhibition of nitrate assimilation, induction of early senescence, and changes to the leaf anatomy and ultrastructure. Results from the literature about CO(2) enrichment indicate that when C(4) plants experience drought in their natural environment, elevated CO(2) concentration alleviates the effect of water stress on plant productivity indirectly via improved soil moisture and plant water status as a result of decreased stomatal conductance and reduced leaf transpiration.It is suggested that there is a limited capacity for photorespiration or the Mehler reaction to act as significant alternative electron sinks under water stress in C(4) photosynthesis. This may explain why C(4) photosynthesis is equally or even more sensitive to water stress than its C(3) counterpart in spite of the greater capacity and water use efficiency of the C(4) photosynthetic pathway.