School-aged children with del22q11.2 tend to show a typical learning and neuropsychological profile, which is characterised by a VIQ-PIQ discrepancy (in favour of the VIQ) and significantly better scores for reading (decoding) and spelling compared to mathematics. To the best of our knowledge, there exists no systematic research on the pre-academic and early academic skills that might underpin these learning difficulties. The purpose of the current study was to investigate more systematically these pre-academic and early academic skills in borderline to normal intelligent (FSIQ > 70) children with del22q11.2 in the last year of kindergarten and first grade of primary school in Flanders. In the kindergarten group, meta-linguistic awareness and counting skills were examined. In the group of first graders, children were tested on reading, spelling and mathematics. Thirteen children (mean age: 6 years 4 months (SD = 0.84); 9 boys, 4 girls) participated in this study. In the present study, there were no differences in intelligence and academic outcomes between boys and girls, and no differences in IQ and academic achievement between children with cardiac defects or severe velopharyngeal insufficiency (VPI) and children without these deficits. With regard to pre-academic achievement in general, a characteristic profile with clearly better results for meta-linguistic awareness in comparison to counting skills was found, but this difference is not statistically significant. Concerning early academic achievement, children with del22q11.2, as a group, perform (despite their somewhat lower general intelligence) on average compared with their age-related peers. However, at an individual level--especially within the domain of counting skills and mathematics--there is a wide variability, with some children showing remarkable learning difficulties already at an early age.