Sebaceous gland hyperplasia (SGH) is a benign condition that originates in the sebaceous follicles and occurs most commonly on the forehead and cheek. It is very rarely found in the genital region; however, a few case reports have described SGH of the vulva. We recently encountered a case of vulvar SGH in a young woman who presented with an asymmetric labium minus and unilateral vulvar hypertrophy. A meticulous histopathological and immunohistochemical examination was performed, and the respective literature was reviewed. The clinicopathological features and immunophenotype of seven previous cases of vulvar SGH were summarized. A careful histopathological examination is required to diagnose vulvar SGH and distinguish it from other vulvar lesions. Given that the increasing incidence of vulvar cancer, it is necessary to biopsy any unusual-appearing lesions on the vulva. Furthermore, it is important to perform histopathological examinations of these lesions to rule out intraepithelial neoplasia or malignancy.