The first part of this article defines the self's conception and development from an organizational perspective. Emphasis is directed to the interplay of biological, caregiving, cognitive, and ecological-environmental factors. Following, discussion turns to a series of studies with children who have Down syndrome to show the effects of biologically based risk factors on early self-development. Then using samples of maltreated children, data are used to reveal the perturbations in self development that arise from problems with attachment relationships and caregiving environments. The concluding section suggests how understanding the self's early development provides insight about maladaptive behavior and psychopathology in self as a consequence of risk. In turn, investigations of high-risk conditions inform us about self and its normal development. Both approaches open up vistas for long-term studies of early self-development.