This study extended follow-up through 2005 of cause-specific mortality in a cohort of 9199 male workers at seven beryllium processing plants, and estimated associations with maximum and cumulative daily weighted average beryllium exposure. We estimated standardized mortality ratios (SMRs) based on US population comparisons for lung, nervous system and urinary tract cancers, chronic obstructive pulmonary disease (COPD), chronic renal disease, and categories containing chronic beryllium disease (CBD) and cor pulmonale. We evaluated associations with maximum and cumulative exposure using internally standardized rate ratios for 5436 workers at the three plants with quantitative exposure information. Overall mortality elevations in the seven-plant cohort were found for lung cancer (SMR ¼ 1.17; 95% CI 1.08, 1.28), COPD (SMR ¼ 1.23; 95% CI 1.13, 1.32), and for the categories containing CBD (SMR ¼ 7.80; 95% CI 6.26, 9.60) and cor pulmonale (SMR ¼ 1.17; 95% CI 1.08, 1.26). Mortality rates for most diseases of a priori interest increased with increasing latency. For the category including CBD, rates were substantially elevated compared to the US population across all exposure groups. Workers with maximum beryllium exposure >/=10 microg/m3 had higher rates of lung cancer, urinary tract cancer, COPD, and the category containing cor pulmonale than workers with lower exposure. Significant positive trends with cumulative exposure were observed for nervous system cancers (p ¼ 0.0006) and, when short-term workers were excluded, lung cancer (p ¼ 0.02), urinary tract cancer (p ¼ 0.003), and COPD (p < 0.0001). Cigarette smoking and exposure to other lung carcinogens are unlikely to explain these elevations.