A variety of systemic inequities place young children from Appalachian communities in the United States at greater risk for poor academic outcomes than children in other parts of the country. Young children with disabilities are at increased risk for poor academic outcomes because of similar inequities and their need for more intensive learning opportunities. The purpose of this study was to examine associations between early literacy skills and home literacy environment of Appalachian preschool children with disabilities. We completed an online literacy screening tool with 57 children between 3 and 6 years of age who had an Individualized Education Program. Parents completed a questionnaire measuring home literacy environment (parenting beliefs and practices). Children's scores on the screening tool were not associated with parent reading beliefs but were associated with modeling writing and communication. Neither parent beliefs nor practices were predictive of children's literacy screening scores. Implications for research and practice are discussed.