RASopathies in their primary meaning are disorders of the RAS-MAP kinase signaling pathway. It is the path through which intracellular signaling takes place, which begins with the binding of ligands to specific receptors, and continues with a series of cascade reactions that ultimately lead to changes in the cell's gene expression. Signal transduction can lead to any of a number of outcomes such as cell differentiation and targeting to a specific cell lineage, proliferation, protein expression, cell cycle arrest and consequent apoptosis. Due to genetic changes in certain segments of the RAS/MAP kinase signaling pathway, phenotypic manifestations of various disorders can occur. Clinical entities that encompass the term RASopathy are neurofibromatosis type I, Noonan syndrome, Legius syndrome, cardiofasciocutaneous (CFC) syndrome, capillary malformation-arteriovenous malformation syndrome (CM-AVM syndrome) and SYNGAP1 intellectual disability. Overall, with a frequency of 1:1000, these are common diseases, although each one rarely appears in the general population. All these disorders share some common, similar characteristics, such as: a tendency to malignant transformations and an increased risk of tumor formation, which is associated with approximately 30% of disorders of this particular signaling pathway. Furthermore, the disorders are characterized by craniofacial deformities that occur with greater or lesser frequency in each individual syndrome. Neurological deficits are common - whether it is a delay in neurological development or intellectual difficulties that vary in severity. What is crucial is to know the genetic basis of each individual RASopathy because of the therapeutic approach to the disease and the action on specific parts of the signaling pathway. Today, three classes of drugs are used for the treatment of RASopathy - RAS activation inhibitors, direct RAS inhibitors and MAPK pathway inhibitors. The effectiveness of these drugs is still being tested with regard to each individual disease that is included under the RASopathy entity, and in addition to the specific method of treatment, research has proven that the time of drug administration, i.e. starting therapy, is also important in order to prevent or alleviate certain sequelae caused by signaling disturbances.