Abstract Objective Prior research on intellectual development in children with congenital heart disease (CHD) yielded inconsistent findings. No study has yet examined the Wechsler Intelligence Scale for Children—Fifth Edition (WISC-V). Given its revised factor structure, it is unknown if the WISC-V performs similarly to prior versions in children with CHD. Methods Twenty children (6-13 years) with CHD received the WISC-V; children with co-morbid neurological illness (i.e., stroke, epilepsy) were excluded. Five children underwent the Fontan procedure. Means, standard deviations, and percent impaired (< 5th percentile) were calculated for composites. Effect size estimates compared children with and without the Fontan procedure. Results Mean age was 10.3 (SD = 2.5; 30% male). The mean Full Scale IQ (FSIQ) was 77.8 (SD = 13.7, range = 46-108). No sex difference in age or IQ were observed. The Visual Spatial Index was the lowest composite (VSI; Mean = 78.7, SD = 18.0), followed by the Fluid Reasoning Index (FRI; Mean = 82.0, SD = 15.6), Working Memory Index (WMI; Mean = 82.4, SD = 16.0), Verbal Comprehension Index (VCI; Mean = 84.80, SD = 15.29), and Processing Speed Index (PSI; Mean = 85.6, SD = 14.6). The FRI was most frequently impaired (50% of sample), followed by WMI (40%), VSI (35%), and VCI/PSI (20%). Although children who underwent the Fontan procedure did not differ in FSIQ (Cohen’s d = .06, negligible effect size), they performed more poorly on the PSI (Cohen’s d = .87, large effect size). Conclusions The WISC-V detects deficits in visual perceptual abilities commonly seen in CHD. However, FSIQ for our sample was lower than previously reported. Children with a history of Fontan procedure may show slower processing speed.