Selective fetal growth restriction (sFGR) has been reported to occur in about 10–15% of monochorionic (MC) twins. The diagnosis of sFGR has been based on variable criteria including estimated fetal weight (EFW), abdominal circumference and/or the degree of fetal weight discordance. Recent studies tend to use a simple definition which includes the presence of an EFW less than the 10th percentile in the smaller twin. Some would argue that the intertwin fetal weight discordance should be included in the definition. Indeed this factor plays a major role in the complications presented by these cases. While the majority of cases with one fetus below the 10th percentile usually will also present with a large intertwin EFW discordance, the contrary is not always true. Thus, it is possible to find MC twins with remarkable intertwin EFW discordance but the EFW of both fetuses are still within normal ranges. Although it appears to be common sense that a large intertwin discrepancy might represent a higher risk for some of the complications described later in this review, there is no consistent evidence to support this notion. Therefore, due to its simplicity, a definition based on an EFW below 10th percentile in one twin is probably the most useful for clinical and research purposes.