Pyloric atresia is a rare condition, comprising less than 1% of all intestinal atresias, with a reported incidence of 1/1,00,000 newborns [1-3]. The usual presenting symptoms include non-bilious vomiting and abdominal distension [3-5]. Delay in diagnosis may occur since the non-bilious vomiting may be attributed mistakenly to gastroesophageal reflux. Complications resulting from such delays include aspiration pneumonia, recurrent lung infections, septicemia, severe metabolic derangements, and gastric perforation [1,2,6]. Here we present a 6-day-old female with pyloric atresia who presented with severe dehydration, hypotension, electrolyte abnormalities, and metabolic acidosis. After clinical stabilization and correction of biochemical abnormalities, the patient underwent surgical correction and had a smooth post-operative course. Currently she is 2 years and 3 months old, and has reached her expected developmental milestones.