Kawasaki disease (KD) is an acute febrile vasculitis that primarily affects children and is associated with systemic inflammation, particularly in the coronary arteries. Coxsackievirus A6 (CVA6) has emerged as a significant agent in atypical presentations of hand, foot, and mouth disease (HFMD), raising the possibility of its involvement in KD. This report presents the case of an 18-month-old Thai boy admitted with symptoms of high fever, sore throat, and ulcerative lesions, initially diagnosed with herpangina. As his condition progressed, additional KD symptoms developed, including conjunctival injection, rash, and elevated inflammatory markers, fulfilling the diagnostic criteria for KD. Notably, throat swab analysis confirmed CVA6 as the causative agent. Phylogenetic analysis revealed that the CVA6 strain closely aligned with Chinese strains from 2023, showing a high nucleotide sequence homology of 98.4%. In conclusion, this case highlights a possible association between CVA6-associated herpangina and KD, suggesting that CVA6 infection may act as a trigger for KD in genetically susceptible children. These findings highlight the need for increased awareness among healthcare providers to promptly identify and manage Kawasaki Disease during peak enterovirus seasons, reducing its impact on children.