Neuroblastoma originates from primitive neural crest cells, located in the adrenal medulla and sympathetic ganglia. Neuroblastoma is one of the leading malignant tumors seen in the perinatal period. It is the most common extracranial tumor in children. Although congenital neuroblastoma usually has a benign course, an agressive clinical outcome is also probable. A three-day-old male patient presented with subcutaneous swellings recognized just after birth. Investigations revealed neuroblastoma. In addition to the left surrenal mass, involvements of liver, bone marrow and subcutaneous tissues were also present. N-myc amplification in tumoral tissue was normal. The patient died due to progressive disease at an early stage of chemotherapy so we could not observe the effect of chemotherapy.