Congenital pyloric atresia is a very rare anomaly. It can occur as an isolated anomaly and can be associated with other conditions such as aplasia cutis congenital/epidermolysis bullosa or multiple intestinal atresias. We present two cases of congenital pyloric atresia, solitary air bubble on X-ray abdomen being pathognomonic sign of pyloric atresia. However, it might present with an intermittent double bubble sign.