Objective Children with neurofibromatosis type 1 (NF1) are at risk for executive functioning (EF) challenges, with little research with preschoolers. Methods EF was examined using parent and teacher ratings of preschool-aged children with NF1 (n = 26) and parent ratings of unaffected children (n = 37) on the Behavior Rating Inventory for Executive Functioning—Preschool Form. Relations to performance on laboratory measures were also examined. Results Based on parent ratings, children with NF1 had more dysfunction than the normative mean on the Working Memory (WM) scale and Emergent Metacognition Index (EMI). Teacher ratings indicated greater dysfunction than the normative mean on the WM and Planning/Organization scales, EMI, and General Executive Composite. Children with NF1 showed more difficulties than unaffected children on the WM scale. Teacher report of WM was significantly correlated with Differential Ability Scales—Second Edition Digits Forward performance. Conclusions WM emerged as an area of difficulty for young children with NF1.

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