"DDX[subscript 3]X" variants are a common cause of intellectual disability (ID) in females, and have been associated with autism spectrum disorder and emotional-behavioural difficulties. In this study, we compared phenotypic data for 23 females with "DDX[subscript 3]X" variants, to 23 females with ID and other genetic diagnoses. We found a wide range of adaptive, social and emotional function within the "DDX[subscript 3]X" group. Autism characteristics did not differ between "DDX[subscript 3]X" and comparison groups, while levels of anxiety and self-injurious behaviour (SIB) were significantly higher in the "DDX[subscript 3]X" group. Within the "DDX[subscript 3]X" group, adaptive function, autism characteristics, anxiety and SIB scores were positively correlated, with evidence for group-specific associations with SIB. Future work is warranted to explore the multilevel mechanisms contributing to social and emotional development in individuals with "DDX[subscript 3]X" variants.