Aim To explore the correlation of serum angiopoietin-1 (Ang-1) and human cartilage glycoprotein-39 (YKL-40) levels with coagulation function and inflammatory response in patients with Kawasaki's disease (KD) complicated with coronary artery lesion (CAL). Methods From January 2018 to December 2,0 children with KD were selected as the study subjects, and 90 healthy children who were examined in this hospital during the same period were selected as the control group. According to whether CAL was combined, they were divided into non-CAL group (69 cases) and CAL group (21 cases), the levels of serum Ang-1, YKL-40, coagulation function, and inflammatory factors were compared between the two groups. Pearson analysis was used to investigate the relationship between serum Ang-1, YKL-40 levels and coagulation function, inflammatory reaction indicators in children with KD combined with CAL. Multivariate Logistic regression analysis was used to identify the factors affecting CAL in children with KD. Results There were significant differences in serum Ang-1 and YKL-40 levels among the control group, the non-CAL group and the CAL group (all P＜0.05). With the increase of the severity of the disease, the serum Ang-1 level in the control group, the non-CAL group and the CAL group decreased gradually, and the YKL-40 level increased gradually (all P＜0.05). Serum Ang-1 was negatively correlated with fibrinogen (FIB), C-reactive protein (CRP), tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6) and white blood cell count (WBC) (all P＜0.05), YKL-40 was positively correlated with FIB, CRP, TNF-α, IL-6 and WBC (all P＜0.05). Multivariate Logistic regression analysis showed that YKL-40 was a risk factor for CAL in children with KD, and Ang-1 was a protective factor (P＜0.05). Conclusion The level of serum Ang-1 decreased and the level of YKL-40 increased in children with KD complicated with CAL, which was correlated with coagulation function and inflammatory response.