The present study evaluated emergency room visit (ERV) risks for all causes and cardiopulmonary diseases associated with temperature and long-lasting extreme temperatures from 2000 to 2009 in four major cities in Taiwan. The city-specific daily average temperatures at the high 95th, 97th, and 99th percentiles, and the low 10th, 5th, and 1st percentiles were defined as extreme heat and cold. A distributed lag non-linear model was used to estimate the cumulative relative risk (RR) of ERV for morbidities associated with temperatures (0 to 3-day lags), extreme heat and cold lasting for 2 to 9 days or longer, and with the annual first extreme heat or cold event after controlling for covariates. Low temperatures were associated with slightly higher ERV risks than high temperatures for circulatory diseases. After accounting for 4-day cumulative temperature effect, the ERV risks for all causes and respiratory diseases were found to be associated with extreme cold at the 5th percentile lasting for >8 days and 1st percentile lasting for >3 days. The annual first extreme cold event of 5th percentile or lower temperatures was also significantly associated with ERV, with RRs ranging from 1.09 to 1.12 for all causes and from 1.15 to 1.26 for respiratory diseases. The annual first extreme heat event of 99th percentile temperature was associated with higher ERV for all causes and circulatory diseases. Annual first extreme temperature event and intensified prolonged extreme cold events are associated with increased ERVs in Taiwan.