Tonne-Kalscheuer syndrome (TOKAS) is a rare disorder with intellectual disability and multiple congenital abnormalities. TOKAS is caused by pathogenic variants in the RLIM gene located on the X-chromosome. Men are affected, while carrier women are usually asymptomatic. Of the 41 patients reported with TOKAS, only 7 antenatal cases were described. We present here 11 antenatal cases, describe the clinical features that made TOKAS recognizable, report novel symptoms and variants, and highlight a recurrent pathogenic variant responsible for most fetal TOKAS cases. This study will facilitate antenatal diagnosis of TOKAS and will enable these families to benefit from appropriate genetic counselling. (https://jmg.bmj.com/content/early/2024/06/07/jmg-2024-109854 )