Congenital juvenile xanthogranuloma (JXG) is an uncommon diagnosis and even more rarely presents with ulceration. We report such a case in a two-week-old girl. Biopsy was performed to rule out any concerning entities. Adequate treatment was provided with topical petrolatum and occasional miconozole or zinc oxide; the mass spontaneously regressed. Because congenital JXG has an excellent prognosis, insight into unique presentations such as this may provide useful information and avoid unnecessary surgical interventions.
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