Kawasaki disease (KD) is an inflammatory vasculitis disorder of unknown etiology. It is a rare but fatal disease and the leading cause of acquired coronary heart disease in children under the age of 5 years. We examined the association of KD with the demographics of family members, parents’ characteristics, and perinatal factors in Taiwanese children. This nested case–control study used data from Taiwan’s Health and Welfare Data Science Center and initially included children born in Taiwan between January 1, 2006, and December 31, 2015 (n = 1,939,449); the children were observed for KD development before the age of 5 years (n = 7870). The control group consisted of children without KD who were matched with each KD case by sex and birth date at a ratio of 8:1. The odds ratio (ORs) of the aforementioned associations were estimated using conditional logistic regression. The risk of KD decreased in children with younger parents [<25 years; younger maternal age, OR = 0.72, 95% confidence interval (CI), 0.66–0.79; younger paternal age, OR = 0.68, 95% CI, 0.59–0.78], lower socioeconomic status, more than 2 siblings (OR = 0.80, 95% CI, 0.73–0.89), and siblings with a history of KD (OR = 4.39, 95% CI, 3.29–5.86). Children living in suburban (OR = 0.95, 95% CI, 0.90–1.00) and rural (OR = 0.81, 95%CI, 0.74–0.90) areas exhibited a lower risk of KD than children living in urban areas. In conclusion, a higher incidence rate of KD was observed in children aged <5 years who had an urban lifestyle, had siblings with KD, were born to older mothers, and belonged to high-income and smaller families. Parental allergic or autoimmune diseases were not associated with the risk of KD.