Melanocytic nevi have been reported in association with several congen
ital syndromes. This review describes the clinical and cutaneous manif
estations of six syndromes associated with congenital melanocytic nevi
, two associated with acquired nevi, and six associated with melanocyt
ic nevi in which insufficient evidence exists to classify them as cong
enital or acquired. It is important to recognize these associations to
evaluate and counsel patients with melanocytic nevi. Early recognitio
n will also facilitate timely intervention.