In adolescence, Kawasaki disease (KD) may present with varied clinical pictures, and the classic clinical criteria are often insufficient to establish the diagnosis. This report presents a clinical case of KD in an adolescent and a brief review of the relevant literature, based on a PubMed search using the terms "adolescence" and "KD". A 13-year-old girl presented with fever, rash, hepatomegaly, and ultrasonographic evidence of abdominal lymphadenopathy. On week 2 after onset of symptoms, the patient developed desquamation of the extremities and thrombocytosis. Echocardiography was normal during the acute illness and remained unchanged at 2-year follow-up. Clinicians should be aware that, in adolescents, KD poses a diagnostic challenge and rarely corresponds to the classic presentation. On retrospective analysis, we believe that abdominal lymphadenopathy should have been recognized as a substitute manifestation of the classic symptom of cervical lymphadenopathy to establish the diagnosis of this fearsome systemic vasculitis syndrome.