Visuospatial deficits have been reported in patients with neurofibromatosis type 1 (NF 1), although detailed observations regarding academic achievement are conflicting. Using neurocognitive testing and magnetic resonance imaging, we studied 12 families, each comprising one child with NF 1, an unaffected sibling of the same age range (6 to 16 years), and both biologic parents. The Full Scale IQ ranged from 70 to 130 among children with NF 1 and from 99 to 139 among unaffected siblings. A significant (p < 0.01) pairwise difference was found between each child with NF 1 and sibling on the Full Scale IQ and the Verbal IQ. On a single visuospatial test, Judgement of Line Orientation, children with NF 1 did significantly worse than siblings (p < 0.01). Children with NF 1 had significant learning disabilities in written language and reading (p < 0.05) and in neuromotor dysfunction (p < 0.005) compared with siblings. A significant correlation was found between the pairwise lowering of the Full Scale IQ and Judgment of Line Orientation scores in children with NF 1 and the number of locations in which, on magnetic resonance imaging, T2-weighted hyperintensities were seen (Full Scale IQ: p < 0.0003; Judgment of Line Orientation score: p < 0.02). We conclude that NF 1 is associated with a significantly lower Full Scale IQ, multifocal cognitive deficits (Verbal IQ, Judgment of Line Orientation score), reading disability, and neuromotor deficit. Pairwise cognitive differences correlated with the number of brain lesions on magnetic resonance imaging.