AbstractBackgroundAcanthosis nigricans (AN) is a cutaneous disorder characterized by symmetric velvety hyperpigmented plaques on intertriginous areas like axilla, neck, inframammary, and groin.AimsTo summarize the pathophysiology and classification of AN, provide an update of diagnostic testing strategies, and describe the current therapeutic options described so far in the literature for this disease.MethodsA comprehensive english language literature search across multiple databases (PubMed, EMBASE, MEDLINE, and Cochrane) for keywords (alone and in combination) was performed. MeSH as well as non‐MeSH terms such as “acanthosis nigricans,” “classification,” “pathophysiology,” “diagnosis,” “treatment,” “topical drugs,” “systemic drugs,” “chemical peeling,” and “lasers” were taken into consideration.ResultsThe pathophysiology of AN revolves around a multifactorial stimulation of proliferation of epidermal keratinocytes and dermal fibroblasts. Various types of AN include benign, obesity‐associated, syndromic, malignant, acral, unilateral, medication‐induced, and mixed‐type. Homeostasis model assessment‐insulin resistance (HOMA‐IR) is a good tool for assessment of insulin resistance. Management involves general measures (weight reduction and addressing the underlying cause, if any), topical drugs (retinoids, vitamin D analogs, and keratolytics), oral drugs (retinoids and insulin sensitizers), chemical peels (trichloroacetic acid), and lasers (Long pulsed alexandrite, fractional 1550‐nm erbium fiber, and CO2).ConclusionAcanthosis nigricans is a treatable condition; however, complete cure and disappearance of lesions are difficult to achieve. Weight reduction is the most scientific and practical management strategy. Long‐term studies and further research is warranted in the pathophysiology and treatment of this common condition.