CNS involvement, except classical lymphocytic meningitis, is exceptionally rare in Kawasaki disease. Herein, we report on two atypical cases of KD with cranial nerve inflammation. The first case presented supranuclear vertical palsy and the second case Bell palsy. Outcome was promptly favorable with IV immunoglobulins, with no long-term complications. No specific KD biomarkers are available and diagnosis of atypical forms is difficult. KD can mimic a systemic illness and appropriate therapy may be delayed.