| Summary: | Acute gastric volvulus in asplenia syndrome is a very rare presentation and only a few cases have been published in the literature. A 10-month-old baby girl with a history of complex congenital cyanotic heart disease presented with a history of repeated episodes of vomiting for 6 days duration. Her ultrasound scan showed a fluid-filled distended stomach, centrally placed liver and absent spleen. The upper gastrointestinal study showed right-sided stomach with mesentero axial volvulus. The gastric volvulus was complicated with a small perforation at the site of the obstruction that was identified during surgery. With the advancement of treatment for sepsis and surgical intervention for cardiac anomalies, the extra-cardiac complications related to other malformations in asplenia syndrome are becoming important issues. It is important to be vigilant about the common associations of asplenia syndrome and the possible rare complications related to visceral malposition.
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