Inflammatory myofibroblastic tumors represent a tumor class of intermediate malignant potential predominantly seen in children and adolescents. Here is the first description of an inflammatory myofibroblastic tumor at the umbilicus of a neonate. In neonates the main sites of presentation are equally distributed between the thoracic and the abdominal region. In a third of the neonates the tumor is identified on an antenatal scan. The preferred treatment option is resection of the tumor. Spontaneous regression has been described.