Objective: This article presents a review of the current diagnosis and treatment of vulvar melanoma in detail to provide useful references for the diagnosis and treatment of the disease in the future. Mechanism: In this review, the author first specifies the topic of the article and the content covered. PubMed was searched for a series of keyword combinations related to the topic, and there was extensive reading of search engine- and database-derived literature from 1970–2022 related to the vulvar melanoma guidelines, including randomized clinical studies, observational studies, and basic research. Based on the published content, the articles were screened according to the year of publication, the number of citations, and the publishing unit. The data and content needed in each article were collected. Finally, the collected data were summarized to provide an in-depth introduction to vulvar melanoma, a rare disease, covering the aspects of clinical features, diagnosis, staging, treatment and prognosis. Findings in Brief: Through this review, we gain a comprehensive understanding of the current diagnosis and treatment of vulvar melanoma and the breakthrough direction of future research. Conclusions: At present, the main diagnostic methods in cases of vulvar melanoma include macroscopic examination, dermoscopy, microscopy, histopathology and imaging examination. The main treatment modalities for vulvar melanoma are surgery, radiotherapy, chemotherapy, immune checkpoint inhibitors, targeted therapy and immune modulators.