Neurofibromatosis type 1 (NF1) is an autosomal dominant disease with an incidence of about 1 in 2500– 3300. Neurological manifestations may be due to involvement of the cerebrum, spine, cranial nerves and peripheral nerves. The severity of NF1 depends on the area of the brain involved. In children they may present with features of failure to thrive, when the lesion involves the hypothalamic area. We are presenting a rare case of diencephalic syndrome with involvement of hypothalamus, chiasm, suprasellar region, midbrain and cerebellum.

Keywords: Neurofibromas Type 1; Diencephalic Syndrome; Failure to Thrive; Optic Nerve Glioma; Unidentified Bright Objects.