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The neuropathologies of the self are a continuum of disorders of the self and identity that occur in the presence of identifiable brain pathology. They include perturbations of the bodily, relational, and narrative self. From the standpoint of neuropsychoanalysis, the most interesting are those that display delusional and confabulatory aspects, especially the delusional misidentification syndromes. Damage to the right, especially medial-frontal and orbitofrontal, sectors is particularly associated with these conditions. According to the ego-disequilibrium theory, in patients with neuropathologies of the self, right frontal damage creates a disturbance in ego boundaries and a breakdown of the observing ego that also facilitates the emergence of developmentally immature styles of thought and ego functioning and the return of “psychologically primitive defenses” such as denial, projection, splitting, and fantasy characteristic of both the adult neurological patients and the child. It is hypothesized that in the course of normal brain development between approximately the ages of 3-8 years there is a movement away from immature defensive functions and fantasies to mature defenses and the inhibition of fantasy that critically depends on maturational processes within the right hemisphere. Once these brain structures are established, the immature defenses and the use of fantasy typical of the child are inhibited and mature adult defenses emerge. In the presence of right frontal damage, however, there is a reemergence of these primitive immature defenses. The hypothesis posits that the preservation and activation of the verbal defenses in these cases, such as verbal denial, projection, splitting, and fantasy, must be the result of the remaining, and presumably relatively intact, left verbal hemisphere and that it even may be possible that the mature defenses are lateralized to the nondominant hemisphere. A four-tiered model of these syndromes is proposed that can broadly account for the origin of all of these conditions. This model emphasizes a multifactorial approach that includes both negative and positive, bottom up and top down, and neuropsychological and psychological factors and explains why any given patient with one of these syndromes may lack a certain feature. Indeed, the model predicts that given that so many interacting factors may play a role in the development of these disorders, we would expect that group studies that examine multiple factors will vary in the presence, importance, and pattern of association and dissociation of any single one.