Aim and objectives To illuminate how people with amyotrophic lateral sclerosis (ALS) create meaning despite the disease. Background Coping strategies for living with ALS have already been investigated. However, there is a lack of studies on how people with the disease find meaning and what helps and hinders this. Design A qualitative descriptive study. Methods Fourteen individual interviews were performed in Spring 2007. The interviews were analysed by qualitative content analysis. Results Two themes emerged to illuminate the complex life situation of the interviewees: experiences of anxiety over life and death and finding meaning despite the illness. It became clear that the uncertain journey towards death was more frightened than death itself. Despite the incurable disease, which brought feelings of life and death anxiety, physical loss, unfairness, guilt, shame and existential loneliness, they also found meaning in life, which strengthened their will to live. Meaning was found through their family and friends, the act of giving and receiving help, the feeling of having a life of their own and accepting the present. The perspective of life was transferred to a deeper view where material things and quarrels were no longer in focus. Conclusions Despite the disease, the participants found meaning in life which strengthened their will to live. Relevance to clinical practiceThe balance between anxiety over life and death and finding meaning in life indicates the importance of support through the whole disease process. Both disease-specific problems and existential questions must be tackled. Nurses and other professionals need to be aware of the patients' existential qualms. There is a need to focus on what is important for the individual, and emphasis must be placed on where that person can find meaning.