Neurofibromatosisis a diseaseconsisting of threegerm layersinvolvedskin, nervous system andorgans of sight. The resultofinheritanceis related to themutationin the geneencoding, which may be spontaneousorassociated with the courseofsuccession.The incidenceis low, one in 3000–4000,000 is affected. Diagnosisof diseaseis based on therecognition of two of the sevensignsadopted by the National Institutesof Health. Neurofibromatosisis an incurable disease. Treatmenttends tocausal treatmentof symptoms. The work is a presentation of a case of 9-year-old boy suffering from neurofibromatosis type 1. Diagnosis was based on the presence of skin lesions similar to “café-au-lait”, freckles around the armpit and groin, and three Lischnodules. At this stage, genetic studies have shown mutation that probably occurred spontaneously. Case study allowed for the identification of problems in accordance with current medical knowledge. Therapeutic nursing procedure action to be taken was described in connection with-existing ailments was described. Nursing care was provided not only the suffering child, but also his family.

Key words: neurofibromatosis, nursing care, therapeutic nursing procedure.