Giant congenital melanocytic nevi are rare with an estimated incidence of approximately 1 in 20,000 live births. They increase the lifetime risk for malignant melanoma and neurological deficits, including leptomeningeal melanocytosis and epilepsy. Recently, we encountered a baby girl in whom giant congenital melanocytic nevi was noted at birth. She presented with large nevus over upper back. MRI and biopsy showed no pathology. Plan was to reassure parents and regular follow-ups. Last follow up was at age of 14 months and the patient is well and achieving her milestones. Here, we describe this rare case with congenital melanocytic nevi to expand the literature about its clinical manifestations, outcomes, risks for malignant melanoma and neurocutaneous melanosis, and possible surgical interventions.