Pigmentary nevi are the most common skin neoplasm found in humans with an incidence of 3 % in whites and 16 % in black infants at birth. The incidence of melanocytic nevi increases throughout infancy and adulthood, as well as at puberty, during pregnancy and with hormonal therapy. Congenital melanocytic nevi (CMN) are defi ned as neural crest derived benign nevomelanocytic proliferations that are present at birth or in the few weeks of life. They are genetically determined and persist throughout life. Prevalence of CMN is 1-2.4 % of newborns; African and Japanese decent have higher incidence than Hispanics or whites and the incidence is 1:500,000. Prevalence of CMN is 1-2.4 % of newborns; African and Japanese decent have a higher incidence than Hispanics or whites and the incidence is 1:500,000. A new classifi cation for determining risks of adverse events in CMN includes the following criteria: satellite nevus count, anatomic localization, color heterogeneity, surface, rugosity, hypertrichosis, and dermal or subcutaneous nodules. They are more frequent on the trunk and extremities, brown-black in color, with small nodules and coarse hair, with a regular smooth and well demarcated border. Satellite melanocytic nevi are common. Proliferative nodules may appear within the nevus, which usually represent benign neurotization of the lesion.By dermatoscopy, the main pattern seen in congenital nevi is the so-called cobblestone pattern, constituted of large, angulated globules, resembling cobblestones. Excision of CMN before the development of malignant melanoma (MM) should be considered to reduce the chances of malignant melanoma development and cosmetic reasons, although it is technically diffi cult, and complete removal is often impossible.