Introduction: Self-care is a crucial aspect in the management of chronic diseases. People with motor neuron disease (MND) live with a chronic degenerative condition in rapid evolution, which leads them to high dependencies. During their illness, they must apply several behaviors including adherence to ventilation and nutritional therapy. Objective: The purpose of this review is to systematically explore the concept of self-care for people with MND. Method: An integrative review of the literature has been conducted. The electronic databases CINAHL, PubMed, and SCOPUS were searched. Results: Fifteen articles met the inclusion criteria and were reviewed; 7 of them discuss decision-making process and adherence to ventilation and nutritional therapy. Five of them discuss strategies used by people with MND to optimize living with this illness. Four of them discuss care and monitoring behaviors performed and recommended to people with MND. No studies focused on self-care in people with MND was identified in the literature. Conclusions: The concept of self-care in people with MND is related to inventiveness, to adaptability, and to dependence. Further studies seem to be needed to understand the concept of self-care in this population.