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The presentation and management of ureterocele has been rarely reported from India and is limited to an odd case report. They can be detected antenatally, may have incidental diagnosis or present with consequences of obstructive uropathy. They always present with secondary complications in adulthood, if the diagnosis and treatment is missed in early years of life. The natural history may be particularly disastrous in cases of bilateral obstructing ureterocele. The complications secondary to obstructive ureterocele can be urinary retention, pyonephrosis, urosepsis, stones and even chronic renal failure. Each of these patients needs an individualized treatment plan. In the period 2003-2013, we managed 36 consecutive patients with varied presentation of this entity. All were managed on the basis of a fixed protocol of investigation and operative intervention. Ultrasound, micturating cystourethrogram, isotope renogram were done preoperatively in all the babies. Those with asymptomatic, unobstructed ureteroceles were left without any intervention. Simple or complex intravesical ureteroceles underwent examination under anesthesia and endoscopic deroofing and DJ stenting for 3 weeks. Of a total of 36 children, 6 were presented with acute complications of ureterocele. They have been managed on an individualized optimum management plan. Their management approach with follow-up is being reported as there is no previous reported series on ureterocele in children from our country.

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A 12-hour-old male infant presented with prolapsed abdominal content through a defect on left side of chest wall with respiratory distress. A thorough clinical examination suggested absence of ectopia cordis, abdominal wall defect, and any bony anomaly. The child expired after 6 hours of admission because of respiratory distress and electrolyte imbalance. Is congenital defect of chest wall associated with diaphragmatic hernia without ectopia cordis and omphalocele, an unusual variant of Cantrell's pentalogy?