Objective To clarify the cognitive profiles including visuo-spatial abilities and social cognition of patients with Kabuki syndrome (KS). Methods Cognitive functions of seven KS patients (8.8±2.1 years, 3 girls), all of whom were confirmed as having KMT2D gene mutation (Out of 7 changes, 3 were nonsense mutations, 1 was frameshift deletion, 1 was splice-site mutation, and 2 were missense changes), were investigated using the Kaufmann assessment battery for children test (K-ABC), with copying tasks of 2D and 3D line figures. We also performed Theory of Mind (ToM) Test (Sally and Anne task). Results The findings from the K-ABC indicated disparities among cognitive areas in the KS participants (p<0.01). Post hoc test (Shaffer's Modified Sequentially Rejective Bonferroni Procedure) revealed that the standard score of the subtest of number recall was significantly higher than those of gestalt closure, triangles and matrix analogies, respectively. In addition, the scores of simultaneous processing scales tended to be lower than those of sequential processing in all the participants and significantly lower in five of the seven participants. The subtests with significantly lower scores than that of the subtest number recall were all grouped into sequential processing. Copying tasks showed the visuo-spatial difficulties often found in patients with Williams syndrome (WS), which is known to have visuo-spatial difficulties. Five out of the seven participants passed the ToM test. Conclusion The K-ABC test revealed significant disparities among the subtests in patients with Kabuki syndrome with the KMT2D gene mutation. They were worse at simultaneous processing tasks and showed visuo-spatial difficulties similar to those of WS patients. Clarifying the characteristic traits of cognitive functions of KS patients may help us to better understand the syndrome.