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Abdominal compartment syndrome secondary to chronic constipation in MECP2 duplication syndrome. Abdominal compartment syndrome (ACS) is an infrequently encountered life-threatening disorder characterised by elevated abdominal pressure with evidence of new organ dysfunction. It is rarely reported in paediatrics. We describe an extremely unusual presentation of a 13-year-old boy with long-standing constipation who developed ACS complicated by refractory septic shock and multiorgan failure. He was treated with emergent decompressive laparotomy and supportive critical care. This case highlights the need for early diagnosis and timely management of ACS to improve its outcome.