A family in which piebaldism is inherited in a dominant pattern is described. In this kindred the cutaneous signs were associated to a high incidence of cancer. Malignancy usually became apparent in the fourth decade and did not correspond to any fixed histopathologic type. Associated cutaneous alterations were: "cafe au lait" spots in two members and telangiectasias in one. A peculiar distribution of blood vessels in fundus oculi was found in one member. One patient had broad nasal bridge and low implanted ears was found in another. The initial cases were two male brothers who had central nervous system tumours. One had a pituitary adenoma and the other a grade III astrocytoma. As it is important to find biologic markers in familial cancer, we speculate, that may be in this family, the marker could be the cutaneous hypopigmentation. Numerous cutaneous syndromes associated to a predisposition to develop cancer have been described, and may be piebaldism is a new one. More observations are needed to corroborate these findings.