The aim of this study was to examine the coping strategies of 49 patients with amyotrophic lateral sclerosis (ALS) and the potential impact of these strategies on survival. A total of 49 subjects were recruited. Each subject was asked to complete a questionnaire on coping strategies called the Brief COPE. The various coping strategies were divided into three factor sets through a factorial analysis: active coping (α 0.84), social support (α 0.71) and avoidance (α 0.71). Each score was divided into two subgroups in relation to the median (limited use vs. frequent use). Equality of survival distributions for the different levels of coping strategies was estimated using the Kaplan-Meier (Log Rank (Mantel-Cox)) model, with adjustment for mental health (GHQ-28), disease severity (ALSFRS), clinical form at onset (bulbar vs. spinal), medical assistance (ventilation and gastrostomy), participation in a clinical trial and gender. Results revealed a significant and positive impact of active coping strategies while taking mental health, ALSFRS scores, clinical form at onset, medical assistance, participation in a clinical trial and gender into account (Log Rank (Mantel-Cox) p < 0.05). Our study clearly demonstrates the impact of active coping strategies on survival in ALS.