A case of familial pseudoatrophoderma colli was found in a 22-year-old Indian woman whose sister and father had similar eruptions. Pseudoatrophoderma colli is an unusual papillary dermatosis in which the lesions appear atrophic. The condition responded very well to treatment with 5% lactic acid ointment. It is likely that pseudoatrophoderma colli is more common than has been reported. It should be considered in the differential diagnosis, along with acanthosis nigricans and confluent and reticulated papillomatosis of Gougerot and Carteaud when a pigmented and papillary dermatosis is present around the neck and upper part of the thorax.