Background Kawasaki disease (KD) is one of the most common systemic vasculitis in children under five years of age. The epidemiology of the disease is not well established in Mexico. The objective of this study was to describe the epidemiology, clinical features and treatment of patients with KD at the Hospital Infantil de Mexico Federico Gomez (HIMFG). Methods We conducted a retrospective, descriptive and analytical study of patients diagnosed from January 2004 to December 2014 with KD at the HIMFG. Results We analyzed 204 cases of which 55% were male, with a median age of 32.5 months (6-120) and a rate of hospitalization of 96%. Twenty percent of patients presented incomplete KD. No differences in the somatometric measurements or vitals were reported. The most frequent symptoms were fever, conjunctivitis (89%), oral changes (84%), pharyngitis (88%) and strawberry tongue (83%). We found higher acute phase reactants in the classic presentation. Echocardiographic alterations were observed in 60 patients (29%) who presented ectasia (12%) and coronary aneurysms (11%). On the other hand, 169 (83%) patients received intravenous immunoglobulin (IVIG), 18 (9%) showed resistance to IVIG, 6 (3%) required corticosteroids, and 2 (1%) infliximab; all received acetylsalicylic acid. Conclusions There were no important differences between classic and incomplete presentations. The incidence of cardiac alterations is less than previously reported in Mexico but similar to other countries.