A case of nevus comedonicus is described which was bilateral, more prominent on the right side, worse on the buttocks with unusual verrucous nodules on the right helix, knee and elbows, and affecting the palms and soles. This patient would appear to have been the most seriously afflicted of the 130 reported cases. In addition to the more comedo‐like lesions on the trunk, a unique feature was the gradual development of verrucous nodules on the helix of the right ear, knee, hand and elbows. The pathologic changes on the nevus comedonicus on the trunk showed the epidermis to be invaginated, in some instances suggesting a tubular‐like quality, with a dilated orifice containing keratin protruding above the surface. Sebaceous glands and hair shaft were absent. The pathologic changes of the verrucous nodule could be described as a more severe form of nevus comedonicus with folding and involution of the epidermis forming large crypts and cysts filled with keratin. Lesions were generally more severe in areas of pressure, which might explain the production of verrucous nodules.