Abstract. Congenital melanoma is a very rare condition defined as a melanoma recognized at or shortly after birth. It may arise de novo or from transplacental metastasis of maternal melanoma. In this case report, de novo primary congenital melanoma presented during the first four months of life, and was diagnosed by histopathological, immunohistochemical, FISH and genetic analysis. Re-excision and sentinel lymph node biopsy was performed, which revealed a micrometastasis in the sentinel lymph node. No adjuvant treatment was provided. There were no signs of relapse with 15 months postoperative follow-up.