Purpose or Case Report: 1. Berdon syndrome, or Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), is a rare condition with heavy morbidity and high mortality. Therefore, recognition and early diagnosis of this entity are critically important for improved patient management and family counselling. Awareness of this rare disease is a prerequisite to early recognition, whether antenatally or in the neonatal period.

2. We present two cases of Berdon syndrome at our institution. Berdon syndrome is a congenital and generally fatal disease characterized by hypoperistalsis of the gastrointestinal system, non-obstructive bladder distension, and microcolon. As of 2018, only 450 cases have been reported in literature since first described in 1976.

3. We present a logical diagnostic approach to this entity from the starting point of megacystis. Starting at megacystis helps narrow the differential diagnoses, with Eagle-Barret syndrome and posterior urethral valves being the main differential considerations.
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