Introduction: A rare kind of epidermal nevus is called nevus comedonicus (NC). It is a part of the nevus comedonicus syndrome, a neurocutaneous condition with symptoms affecting the eyes, the skeleton, and the nervous system. Histopathology and clinical examination are used to make diagnoses. There is new information on signaling pathways in illnesses linked to acne that may also affect NC. Contrary to acne-related comedones, the epithelium is frequently hyperkeratotic and occasionally acanthotic, making comedo extraction more difficult. Dermatologists need to be aware of any potential connections between NC and extracutaneous findings like NCS and skin cancers. Complete surgical excision is the best treatment option. Future advancements in laser technology could make them an option. Targeted therapy will become an option in the future when molecular etiology is more understood. Better patient outcomes and a higher quality of life may result from the proper diagnosis and treatment of nevus comedonicus. Aim of the study: The present review describes nevus comedonicus examination and therapy and briefly explains the function of the interprofessional team in treating individuals with this condition. Methodology: The review is a comprehensive research of PUBMED from the year 1895 to 2018 Conclusion: Any patient presenting with lesions indicative of nevus comedonicus should be checked for signs of nevus comedonicus syndrome. Patients need to be warned about the likelihood of sporadic flares. Although there are currently no clear randomized control trials that can predict therapeutic success with any certain modality, patients must be made aware of all of their alternatives in order to make an educated choice. Keywords:Nevus comedonicus, Epidermal nevus syndrome, Tyrosine kinase receptors, Rare skin disorders,