Summary: Neurofibromatosis type 1 (NF1) is a rare genetic disorder inherited in an autosomal dominant pattern. Familial occurrence is observed in approximately 50% of cases, while the remainder result from de novo mutations affecting the large NF1 gene located on chromosome 17. The disease has a highly variable and age-dependent clinical expression, which creates diagnostic challenges – particularly in the pediatric population – due to its phenotypic heterogeneity. According to the revised diagnostic criteria published in 2021, a diagnosis of NF1 requires at least two of the following: 1) six or more café au lait macules (above 0.5 cm in prepubertal and above 1.5 cm in postpubertal individuals); 2) axillary or inguinal freckling; 3) two or more neurofibromas or one plexiform neurofibroma (PNF); 4) optic pathway glioma; 5) two or more Lisch nodules or choroidal abnormalities; 6) distinctive osseous lesions (e.g., sphenoid wing dysplasia, pseudarthrosis); 7) a first-degree relative with clinically confirmed NF1; 8) a heterozygous pathogenic variant in the NF1 gene. A major clinical concern in NF1 is the occurrence of benign and malignant tumors. Among benign neoplasms, plexiform neurofibromas (PNFs) pose the greatest therapeutic challenge due to their potential to cause chronic pain, functional impairment, and in some cases, life- -threatening complications resulting from their anatomical location or malignant transformation into MPNSTs. As of 2024, patients in Poland with inoperable, symptomatic PNFs may receive selumetinib, a MEK inhibitor, as part of the national drug program B.155, following qualification by a designated Multidisciplinary Coordination Team. Given the multisystemic and progressive nature of NF1, affected individuals require lifelong multidisciplinary care. In response, Centers for Coordinated Medical Care (CKOM) for NF1 and related RASopathies have been established in Poland since 2020, aiming to improve diagnosis, access to specialized therapies, and overall patient outcomes.