Background: People with chronic illnesses may struggle to adapt psychologically to the illness experience and have feelings of identity loss, self-diminishment, and biographical disruption. This may limit people’s ability to engage in optimal self-management. Systemic sclerosis is a debilitating, stigmatizing, and life-limiting progressive chronic illness with significant disfiguring effects. Little is known about the identity management process in people with disfiguring and debilitating conditions such as systemic sclerosis. Purpose: The purpose of this study was to generate a grounded theory explicating the process of maintaining a sense of self in people living with systemic sclerosis. Methods: Fifteen women with systemic sclerosis were recruited to ensure representation of a range of illness duration and progression. Semi-structured interviews were conducted, transcribed, and analyzed using open, selective, and theoretical coding. Results: A basic social process of “maintaining self” was generated from the data that explained the women’s experience of living with systemic sclerosis and how they tried to hold on to their identity. Three core categories were identified. Adapting to changes are the behaviors that participants struggled through to carry on with their everyday lives. Dismantling of self was a distressing internal process where participants lost their sense of self and purpose. Restoring self was a transformative process that allowed participants to rewrite and rebuild their biographies. Conclusions: Findings suggest that the management of identity was important for understanding how people adapt to life with systemic sclerosis. This study can help nurses better understand how to support patients holistically with the management of systemic sclerosis.