[2]: 867  Common characteristics include a congenital white forelock, scattered normal pigmented and hypopigmented macules and a triangular shaped depigmented patch on the forehead.

The condition is very common in mice, rabbits, dogs, sheep, deer, cattle and horses—where selective breeding has increased the incidence of the mutation—but occurs among chimpanzees and other primates only as rarely as among humans.

Piebaldism differs from albinism in that the affected cells maintain the ability to produce pigment but have that specific function turned off.

Human piebaldism has been observed to be associated with a very wide range and varying degrees of endocrine disorders, and is occasionally found together with heterochromia of the irises, congenital deafness, or incomplete gastrointestinal tract development, possibly all with the common cause of premature cutting off of human fetal growth hormone during gestation.

Piebaldism is an autosomal dominant[4] hereditary condition, which tends to produce high rates of inheritance and long chains of generational transmission.