Understanding the causes and places of death of amyotrophic lateral sclerosis (ALS) patients leads to the development of better treatment modalities. The present study aimed to identify the causes and places of death of ALS patients in south-west China. A total of 139 ALS patients (89 males and 50 females) were regularly followed up in the Department of Neurology, West China Hospital of Sichuan University from 2004 to 2010 until their deaths. Information on the causes and places of death was provided by family members, caregivers, or family physicians. Overall, 91 patients (65.5%) died of respiratory failure, 36 patients (25.9%) died of nutritional causes due to dysphagia, five patients (3.6%) met sudden deaths, three patients (2.2%) died of heart related causes, and four patients (2.9%) died of unknown reasons. Of the 139 patients, 114 (82%) died at home. Consistent with reports in the literature, respiratory failure is the leading cause of death in ALS patients. However, unlike in other studies, the second leading cause is nutrition related. Most patients die at home. The inconsistencies of these results with foreign studies may be attributable to economic and cultural differences. The findings have significant implications on the treatment modalities and palliative care for ALS patients in China.