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Children with developmental delays have a much higher incidence of behavior problems than do children who are typically developing. This article reviews the current research on the occurrence and nature of behavior problems in these children, with particular attention to issues relevant to young children and the type and severity of problems that have been observed at ages as young as 2 years. Evidence in support of a conceptual framework for understanding how such behavior problems may develop is presented, with a particular focus on early biological or constitutional factors, family stress, and children's developing self-regulatory capacities. Implications for practice are discussed.