Paradoxically, individuals with autism spectrum conditions have been characterized as both impaired in self-referential cognitive processing, yet also egocentric. How can the self in autism be both ‘absent’ (i.e., impaired self-referential cognition), yet ‘all too present’ (i.e., egocentric)? In this paper, we first review evidence in support of both claims. Second, we highlight new evidence illustrating atypical function of neural systems underlying self-representation in autism. We suggest that egocentrism and impaired self-referential cognition are not independent phenomena. Instead, both egocentrism and impaired self-referential cognition in autism can be resolved as expressions of one common mechanism linked to the atypical function of neural circuitry coding for self-relevant information. We discuss how autism provides a unique window into the neurodevelopmental mechanisms enabling a critical developmental transition in self-awareness. This transition involves a dual understanding that one is similar to, yet distinct from others. The neural and cognitive basis of this developmental transition is central to understanding the development of social cognition as well as the paradox of the autistic self and its relation to social impairment in autism. Copyright © 2010 John Wiley & Sons, Ltd. For further resources related to this article, please visit the WIREs website.