Neurofibromatosis type 1 is characterized on magnetic resonance imaging by optic nerve gliomas, parenchymal gliomas, and foci of prolonged T2 relaxation involving the brainstem, cerebellum, midbrain, internal capsule, and basal ganglia. We report a child with neurofibromatosis type 1 in whom serial magnetic resonance imaging demonstrates spontaneous regression of basal ganglia lesions. These lesions, characterized on magnetic resonance imaging by increased signal intensity on T1- and T2-weighted sequences, involved the globus pallidus and internal capsules in a bilateral fashion.