Birthmarks in newborns are common and cause great anxiety in the parents. Developmental defects include malformations, deformations and disruptions. Nevi are nonneoplastic proliferations of tissue, usually present at or soon after birth. They are broadly classifies according to the tissue of origin into epidermal, melanocytic, dermal, subcutaneous and vascular nevi. As many nevi have associated developmental defects, a multidisciplinary examination to rule out neurological, ocular, skeletal and cardiovascular complications may be required. Many of these nevi do not require active management except for cosmetic reasons. Those nevi that cause functional impairment such as vascular nevi or complications like malignancy require active intervention at an early stage. Large congenital melanocytic nevi require evaluation for removal, whereas smaller nevi may be observed for malignant changes. High- and intermediate-risk skin markers of spinal dysraphism such as dermal sinuses, tails, atypical dimples require evaluation with magnetic resonance imaging or ultrasonography. Pediatricians should be familiar with various birthmarks and be comfortable discussing disease prevention and cosmetic strategies.