Vaginal primary malignant melanoma is an exceptionally rare, especially in Taiwan, yet lethal disease, with a five-year overall survival rate of only 18%. Recent elucidation of alternating pathogenesis and refinement of diagnostic methodologies are vital for future management standardization. The authors describe a case of a 58-year-old postmenopausal Chinese woman with vaginal malignant melanoma, with vaginal spotting and vulvar itching as initial presentation. After immunohistochemical staining of biopsy specimen had shown positive of S-100 and HMB-45, and total vaginectomy with bilateral inguinal lymphadenectomy was performed. The patient remains free of disease six months after discharge. In conclusion, vaginal primary malignant melanoma with initial presentation mimicking vaginitis and symptomatic menopausal state could cause diagnostic confusion, especially at postmenopausal woman. By understanding clinical presentation, pathogenesis, differential diagnosis, and treatment options of vaginal primary malignant melanoma, effective diagnosis could be possible.
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