Hypertrophic pyloric stenosis (HPS) is a common condition affecting infants that presents with progressive projectile nonbilious vomiting. The pyloric portion of the stomach becomes abnormally thickened and manifests as gastric outlet obstruction. The cause is unknown. Pyloromyotomy remains the standard of treatment and outcome is excellent. This article reviews the diagnostic work up and imaging, preoperative resuscitation, the various surgical approaches used, and the effect of subspecialty training on outcomes after pyloromyotomy. Postoperative care and the variety of postoperative feeding regimens applied after pyloromyotomy are reviewed, as well as intra- and postoperative complications. Finally, medical management, in lieu of surgery, is discussed.