Patient: 7-year-old healthy, Caucasian male, BCVA 1.0 at OD, 1.0 at OS. Ocular Medical History: during routine eye examination were diagnosed multiple, unilateral Lisch nodules. A thorough history and physical examination revealed signs of NF1 on skin and in brain. General Medical History: : pigmentation abnormalities at hand and arm. Main Complaint: no. Methods: Colour Photography Anterior Segment,Brain-MRI, Skin Photography. Colour Photography Anterior Segment: several Lisch nodules (melanocytic hamartomas of the iris). NMR brain: focal high signal intensities on T2-weighted images in the deep cerebral gray matter and in the cerebral white matter. Skin: pigmentation abnormalities- café au lait macules. Colour Photography Anterior Segment of his Mother: showinmg an amelanocytic hamartoma of the iris at 12h. Discussion: Neurofibromatosis type 1 (NF1) called also von Recklinghausen's disease is an autosomal dominant genetic disorder with a  complex clinical course. An important diagnostic criterion of the disease, is the presence of Lisch nodules- hamartomatic changes of the iris. The presence of two or more Lisch nodules (melanocytic hamartomas of the iris) is one of seven diagnostic criteria for neurofibromatosis type 1 (NF1), a common monogenic disorder of dysregulated neurocutaneous growth. Lisch nodules associated with Neurofibromatosis Type 1 (NF1) are usually multiple and bilateral in nature. Bikowska-Opalach et al. (1) described clinical features and molecular mechanism of Neurofibromatosis type 1 (NF1). They reported that symptoms concern mainly skin with pigmentation abnormalities with café au lait macules, and central nervous system (cognitive impairment, epilepsy, attention deficit hyperactivity disorder and gliomas). Pathologic changes may also affect other organs and systems, including skeletal system (scoliosis, hypostature, osteoporosis, pseudoarthrosis and sphenoid wing dysplasia) or cardiovascular system (hypertension, inherited cardiovascular malformations). The development of NF1 is a consequence of inactivation of NF1 gene. The gene, located on chromosome 17, has one of the greatest frequencies of spontaneous mutation in the whole human genome. Gene product, a cytoplasmic protein called neurofibromin, is a tumor suppressor, with expression detected in various cells, mainly in malanocytes, neurons, Schwann cells and glial cells. Due to its anti-tumoral function, inactivation of NF1 protein leads to the growth of several neoplasms, concerning mainly skin and central nervous system (CNS). Skin tumors are actually malignances of the peripheral nervous system (PNS) and include cutaneous, subcutaneous and plexiform neurofibromas. In the CNS the most frequently occurring tumors are gliomas located in the optic pathway. Histologically, CNS tumors are usually a  benign pilocytic astrocytoma, consisting of malignant-transformed astrocytes. Boley et al. (2) reported that Lisch nodules arise secondary to exposure to ultraviolet (UV) radiation from sunlight.   Literature: (1) Bikowska-Opalach B, Jackowska T.Neurofibromatosis type 1 - description of clinical features and molecular mechanism of the disease. Med Wieku Rozwoj. 2013 Oct-Dec;17(4):334-40. (2) Boley, S; Sloan, JL; Pemov, A; Stewart, DR. A quantitative assessment of the burden and distribution of Lisch nodules in adults with neurofibromatosis type 1. Invest. Ophthalmol. Vis. Sci., 2009 vol. 50(11) pp. 5035-43