Dowling-Degos disease is a rare genodermatosis characterized principally by progressive reticulate hyperpigmentation of the flexures. Although the condition is benign, it may be associated with skin malignancies. Furthermore, the characteristic skin changes may be psychologically and socially detrimental to the patient as a result of the unsightliness of the condition. The present report describes a case in which this disease was associated with a keratoacanthoma, and summarizes current concepts on this skin disorder.