Dowling-Degos disease is a rare autosomal dominant inherited pigmentary disorder, mostly confined to the flexures. Diagnosis is established based on the clinical and histopathological correlation. The authors describe the clinical case of a female patient with vulvar involvement and multiple seborrhoeic keratoses on her face, neck, and upper trunk. Major and minor clinical manifestations of Dowling-Degos disease are discussed, with particular emphasis on the genital location of the lesions, which is a rare finding. Also the presence of seborrhoeic keratosis is discussed as a coincidence or a true-associated phenomenon.