Birthmarks are visible skin lesions present at birth or shortly thereafter, that can be frightening to parents. Birthmarks can be red, blue, brown-black, or white; flat or raised; hairy or warty and occur as a single or multiple lesions or distributed over a segment of the body. Fortunately, regardless of color or shape, the vast majority of congenital skin lesions or “birthmarks” are a benign, isolated finding and parents can be reassured.

Occasionally, a birthmark may be a sign of an associated congenital defect or an underlying syndrome. The location and morphology of a birthmark can help determine the risk of an association and direct the appropriate workup.

It is important to quickly differentiate a worrisome birthmark from an insignificant lesion and reassure parents as soon as possible. However, even benign and medically insignificant birthmarks can be of great cosmetic significance and require treatment. This chapter will discuss the most common birthmarks, their potential associations and the necessary workup and management.