<p>Abdominal compartment syndrome is a life-threatening condition caused by elevated intra-abdominal pressure, leading to impaired organ perfusion and dysfunction. In pediatric patients—especially neonates—diagnosis is challenging due to non-specific signs and limited clinical awareness. Pediatric abdominal compartment syndrome remains underrepresented in the literature, with few published cases and no standardized guidelines for diagnosis or management in this age group. We report the case of a premature newborn from a twin pregnancy who developed abdominal compartment syndrome following surgical repair of an omphalocele, emphasizing the value of ultrasound as an effective tool for the diagnosis and monitoring of abdominal compartment syndrome. This report contributes to the limited evidence available, highlighting the need for vigilance in similar high-risk scenarios, as early recognition and timely intervention are crucial to prevent irreversible damage.</p>