Background Large and rapidly growing abdominal tumors may result in fatal outcomes in newborns. In some cases, a rapidly worsening clinical condition requires surgical decision‐making despite the absence of a precise histological diagnosis. In these situations, there is neither a guide nor consensus. Case We highlight our experience with five patients with large abdominal tumors and assess the available literature for the best possible management of a rare condition. Conclusion In these cases, laparostomy should be considered as a life‐saving procedure. If the liver is involved and coagulopathy is present, prognosis is often compromised.

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.