Infantile hypertrophic pyloric stenosis is a common condition affecting young infants; despite its frequency, it has been recognized only for a little over a century, and its etiology remains unknown. Nevertheless, understanding of the condition and of effective treatment have undergone a remarkable evolution in the 20th century, reducing the mortality rate from over 50% to nearly 0%. The lesion is characterized by gastric outlet obstruction and multiple anatomic abnormalities of the pyloric antrum. The antropyloric muscle is abnormally thickened and innervated, and the intervening lumen is obstructed by crowded and redundant mucosa. Recognition of the obstructive role of the mucosa led to discovery of effective surgical treatment. Accurate clinical diagnosis in patients in whom a thickened antropyloric muscle is not readily palpable can be difficult, resulting in delayed diagnosis and can lead to emaciation and electrolyte imbalance, making the patient a suboptimal surgical candidate. Current imaging techniques, particularly sonography, are noninvasive and accurate for identification of infantile hypertrophic pyloric stenosis. Successful imaging requires understanding of anatomic changes that occur in patients with this condition and plays an integral role in patient care. Accurate, rapid, noninvasive imaging techniques facilitate rapid referral of vomiting infants and prompt surgical treatment of more suitable surgical candidates. Copyright RSNA, 2003