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Abstract 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.