Hereditary sensory and autonomic neuropathy type IV [HSAN IV] is a rare ge-netic disorder of children characterized by the inability to feel pain and to sweat [anhidrosis]. There is insensitivity to temperature. Dysfunction of the sensory nerves which are responsible for pain and temperature causes the sensory loss. NTRK1 gene variants cause this disorder, inherited in an autosomal recessive pattern. This review aims to synthesize current research on the clinical, molecular, and diagnostic aspects of Hereditary Sensory and Autonomic Neuropathy -IV [HSAN IV] and identify gaps for future research. The objectives include reviewing studies on HSAN's clinical features and genetic variations, assessing the effectiveness of diagnostic methods, genetic testing and highlighting inconsist-encies in genotype-phenotype correlations. We searched PubMed, Scopus, Web of Science, Embase and Google Scholar using terms 'HSAN' and 'genetic vari-ants', with no time frame restrictions. The literature on the clinical and molec-ular characteristics, diagnostics, therapeutics including recent advances of HSAN IV has been reviewed. This review highlights advancements in understanding HSAN's clinical and genetic aspects, while emphasizing the need for further re-search to address gaps in genetic knowledge and improve diagnosis and treatment outcomes.