Large and rapidly growing abdominal tumors endanger vital prognosis in neonates. Sometimes, quickly worsening patients' clinical conditions demand surgical decision-making that cannot wait for precise histological diagnosis. There is neither consensus nor rules to manage these situations. We presented our experience with five patients and we looked in the literature for the best possible management of such rare disease. Laparostomy should be considered only as a safe-life procedure and if the liver is affected by the tumor and a coagulopathy is present, the prognosis is often compromised.