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OBJECTIVE: To determine incidence, geographic distribution, and seasonal variation of IDDM in children 0-14 years of age living in Puerto Rico. Because these data have been collected through the infrastructure of the World Health Organization's DiaMond project, these results are directly comparable with incidence data from other population worldwide involved in this study. RESEARCH DESIGN AND METHODS: Beginning in 1990, new cases of IDDM were registered retrospectively from 1985 and prospectively to 1994 by review of medical records from island hospitals. Included in the hospital registry are 1,527 cases of IDDM. Validation of the primary source was by three secondary lists of cases obtained through diabetic camps, surveys of schools, and a government registry. Long linear modeling (capture-recapture) was used to correct incidence. RESULTS: Mean incidence of IDDM from 1985-1994 was 18.0 cases/100,000 children per year (95% CI 17.6-18.3). There was a slight female rather than male predominance: 51% of the cases were girls, and 49% were boys. Although Puerto Rico has marked variation in rainfall, altitude, and genetic markers, no significant differences are found in the incidence rates of different areas or seasons of the island. CONCLUSIONS: This registry of Puerto Rican children is the largest IDDM registry of minority children in the U.S. The results of this study indicate that the annual incidence of IDDM of children living in Puerto Rico is higher than the incidence of other multiracial ethnic groups living in the U.S.