Congenital nevi are present in approximately 2-3% of neonates. These lesions are present at birth. They are characterized by pigmented lesions with regular margins, smooth or lobular surfaces and occasionally have long coarse hair. The risk of melanoma development is proportional to the size, especially if it involves over 5% of the body surface, or is > 20 cm in adolescents (large/giant congenital nevus). The risk of malignant change ranges from 5-40%. We present a case of a congenital melanocytic nevus in a six-year-old female child which was surgically treated.