Hypertrophic pyloroduodenal tuberculosis is a rare cause of Gastric Outlet Obstruction (GOO) often forgotten in the differential diagnosis of gastric outlet obstruction. Since laboratory and radiological investigations often prove inconclusive in reaching the diagnosis of hypertrophic pyloroduodenal tuberculosis, surgery has a key role in the management of hypertrophic pyloroduodenal tuberculosis. Postoperative anti-tuberculosis chemotherapy (ATT) becomes imperative for complete resolution of hypertrophic pyloroduodenal tuberculosis. This case report describes the condition and management in a young girl.