The article provides a comprehensive overview of the diagnosis and management of neurofibromatosis type 1 (NF1), a common neurocutaneous disorder with autosomal dominant inheritance. It outlines the diagnostic criteria, major differential diagnoses, clinical manifestations, and management strategies for NF1. The guidelines are based on clinical studies and expert consensus, emphasizing the importance of early diagnosis and multidisciplinary care. NF1 is characterized by features such as café au lait spots, neurofibromas, and Lisch nodules, with a birth incidence of 1 in 2500 to 1 in 3000. The *NF1* gene, located on chromosome 17q11.2, is responsible for the condition, and mutations in this gene lead to a loss of neurofibromin, a tumor suppressor protein. Diagnosis requires at least two of the clinical features listed, with some exceptions for mosaic or segmental NF1. The article also discusses the differential diagnoses, including other forms of neurofibromatosis and conditions that may resemble NF1. Management involves regular monitoring, education, and specialist care, with a focus on early detection of complications such as malignant peripheral nerve sheath tumors (MPNST), optic pathway gliomas, and spinal cord compression. The article highlights the importance of genetic counseling, prenatal diagnosis, and the role of specialist clinics in providing coordinated care. It also addresses various aspects of NF1, including psychological, orthopedic, cardiovascular, gastrointestinal, and neurological complications, along with the impact of the condition on quality of life and the need for multidisciplinary support. The guidelines emphasize the importance of a team approach involving neurologists, geneticists, dermatologists, and other specialists to ensure effective management and improve outcomes for individuals with NF1.The article provides a comprehensive overview of the diagnosis and management of neurofibromatosis type 1 (NF1), a common neurocutaneous disorder with autosomal dominant inheritance. It outlines the diagnostic criteria, major differential diagnoses, clinical manifestations, and management strategies for NF1. The guidelines are based on clinical studies and expert consensus, emphasizing the importance of early diagnosis and multidisciplinary care. NF1 is characterized by features such as café au lait spots, neurofibromas, and Lisch nodules, with a birth incidence of 1 in 2500 to 1 in 3000. The *NF1* gene, located on chromosome 17q11.2, is responsible for the condition, and mutations in this gene lead to a loss of neurofibromin, a tumor suppressor protein. Diagnosis requires at least two of the clinical features listed, with some exceptions for mosaic or segmental NF1. The article also discusses the differential diagnoses, including other forms of neurofibromatosis and conditions that may resemble NF1. Management involves regular monitoring, education, and specialist care, with a focus on early detection of complications such as malignant peripheral nerve sheath tumors (MPNST), optic pathway gliomas, and spinal cord compression. The article highlights the importance of genetic counseling, prenatal diagnosis, and the role of specialist clinics in providing coordinated care. It also addresses various aspects of NF1, including psychological, orthopedic, cardiovascular, gastrointestinal, and neurological complications, along with the impact of the condition on quality of life and the need for multidisciplinary support. The guidelines emphasize the importance of a team approach involving neurologists, geneticists, dermatologists, and other specialists to ensure effective management and improve outcomes for individuals with NF1.