The aim of the study was to summarize literature data on oral and maxillofacial manifestations of neurofibromatosis I (NFI) and to analyze clinical case in with dentist had the leading role in proper diagnosis of the disease. Literature review showed main oral alterations in NFI to be: neurofibroma formation seen in 8-14% of children and adolescents, dysplastic «orthodontic» phenotype with shortened mandible, maxilla and sphenoid bones; radiologic signs including alveolar nerve canal and mental foramen widening in almost one third of patients; lesions of cranial nerves (mainly trigeminal, facial and glossopharyngeal), with non-specific paralysis clinic. These symptoms, however, develop gradually throughout the life and may be evident only in late adolescents. The most pathagnomic sigh is café-au-lait spots as they present in 95% of children at birth. The described clinical case demonstrates the difficulties in diagnostic of NFI in children in the absence of mutilating plexiform neurofibromas. The disease provoked significant functional disorders in maxillofacial area which resulted not only in lower quality of life but also mimicked iatrogenic complications of routine dental treatment.