| Summary: | Gastric Outlet Obstruction (GOO) is largely attributed to Infantile Hypertrophic Pyloric Stenosis (HPS) in the pediatric population with an incidence of 1–3 per 1000 live births and is rare in females with a male-to-female ratio of 4:1. However, there has been a rise in reports of cases of GOO in childhood which is considered a rare condition with an incidence of 1:100,000. The association of pyloric stenosis presenting concurrently with malrotation is much rarer in pediatric literature with a paucity of reportage on it. Within the childhood population, various factors have been attributed to acquired forms of GOO. Notable among them are products of consanguineous marriage and cow's milk protein allergy (CMPA).We present what we believe is the first reported case of primary acquired GOO in childhood occurring with malrotation in a 4-year-old female, who had a long history of intake of unpasteurized cow milk and is a product of consanguineous parents.
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