Congenital melanocytic nevi (CMN) are defined as nevi present at birth or appeared within the first year of life and persistent throughout life. Categories include small (<1.5 cm), medium (1.5–20 cm), and large or giant (>20 cm). CMN are usually larger than common nevi with color and shape variegation and tendency to become palpable and covered by terminal hair. The risk of melanoma development is related to the size of CMN and the age of patients with a tendency to develop earlier and more frequently in giant CMNs. At dermatoscopy examination, the typical pattern of CMN is globular and defined as a “cobblestone pattern,” characterized by the presence of large and angulated globules resembling cobblestones. The “globular pattern” is most frequently found in the CMN of the head, neck, and trunk, while a “reticulated” or “mixed pattern” (reticular–globular) can be detected especially at the extremities. Atypical dots or globules, which are common in CMN of all sizes, serve as indicators of potentially concerning changes within the lesions.