Purpose: Increased risk of death has been reported in patients with intractable epilepsy (IE) taking nitrazepam (NZP). Methods: Between January 1983 and March 1994, 302 patients with IE were entered into a NZP compassionate‐plea protocol. NZP was discontinued if there was <50% seizure reduction or significant side effects. In some patients with >50% reduction, it also was discontinued for lack of sufficient effect. At the end of follow‐up for this study, 62 patients remained taking NZP. Patients took NZP from 3 days to 10 years. Results: Twenty‐one of 302 patients died after institution of NZP. Fourteen of 21 of these were taking NZP at death, and in five of 21, the NZP had been discontinued. Two patients were excluded from analysis, because it is unclear whether NZP had been discontinued before death. Six other patients were lost from follow‐up. Of the 14 deaths with NZP, seven were sudden, six were of pneumonia, and one was of cystinosis. Nine had at least one contributing factor, such as dysphagia, gastro‐esophageal reflux, or recurrent aspirations. The 294 patients took NZP for a total of 704 patient years (ptyrs), and were discontinued for a total of 856 ptyrs. There were 1.98 deaths/100 ptyrs on NZP compared with 0.58 deaths/100 ptyrs without NZP, most of the former being associated with side effects of NZP. Mortality in patients younger than 3.4 years was 3.98 with NZP compared with 0.26 deaths/100 ptyrs without NZP (p = 0.0002). Corresponding figures in patients 3.4 years or older were 0.50 and 0.86 deaths/100 ptyrs, respectively. Conclusions: NZP therapy for epilepsy apparently increases the risk of death, especially in young patients with IE. This should be considered in antiepileptic drug (AED) management decisions.