Sternal defect is a rare congenital malformation and sporadically developed by ventral fusion defect at an early stage of the embryogenesis. This is an isolated defect more commonly and can be unusually associated with other abnormalities. Sternal malformation/ vascular dysplasia association was first reported by Hersh et al. in 1985 and has not yet in Korea. The clinical features are typically cleft of the sternum that is covered with atrophic skin, a median abdominal raphe extending from the sternal defect to umbilicus and cutaneous craniofacial hemangiomata. This spectrum has been occasionally described as an overlap with PHACE syndrome and is needed to evaluate brain, heart, and eyes. We demonstrate a full-term newborn girl presented with skin defect over the sternum and supraumbilical raphe, who had not anomalies in other sites. Multiple hemangiomas were subsequently appeared on her chin and upper chest wall, and respiratory distress due to subglottic hemangioma was developed within the first 2 months of life. Her symptoms have been controlled by administration of oral prednisolone.