In 1976, Berdon et al. first described five newborn girls presenting with small intestinal obstruction, microcolon, and megacystis (Berdon et al. 1976). They observed that the enteric smooth muscles of these patients failed to contract despite normal ganglion cells throughout the narrow and dilated segments. MMIHS is a rare and severe congenital syndrome characterized by abdominal distention, which is mainly due to an enlarged unobstructed urinary bladder, a small intestine (microcolon), and absent or reduced intestinal peristalsis (functional gastrointestinal obstruction). Other presenting manifestations include bilious vomiting, failure to pass meconium, and inability in voiding. The prevalence of this syndrome remains to be unknown; however, a female to male predominance has been reported in studies. Berdon syndrome is generally considered a disorder of neonates; however, two cases of familial visceral myopathy, a disorder of this phenotypic spectrum, were reported with an adulthood-onset (Lehtonen et al. 2012; Holla et al. 2014).