This is a descriptive study of the occurrence of Kawasaki disease (KD) in an urban population that was a majority of African Americans. Records of 189 children (mean age, 2.9 ± 2.2 years [range: 2 months to 11.1 years]) hospitalized for KD over 8 years (January 1, 1992 to December 31, 1999) were reviewed and data analyzed. One hundred thirty-six (72%) were African American (AA), 43 (23%) were white, and 9 (5%) children were "others." The annual frequency was 15 for AA and 7.7 for white per 100,000 5-year-old children. Coronary artery abnormalities (CAA) were reported in 21 (11%) children (18 [13.2%] of 136 AA, and 3 [4.7%] of 43 whites [p=0.095]). AA children with CAA were older than their white counterparts (26 to 24 vs. 5 to 2.8 months, p=0.03). There was a higher occurrence in winter and spring (110 cases [58%] vs. 79 cases [42%]) compared to summer and fall. KD occurrence was positively associated with average monthly snowfall (r=0.35, p=0.004) and inversely associated with average monthly temperature (r= - 0.2, p=0.048). African-American children were more likely to be hospitalized for KD compared to white children. The association of KD with temperature and precipitation suggest that it is influenced by environmental factors.