Megacystis-microcolon-intestinal hypoperistalsis syndrome is a rare, congenital, autosomal recessive syndrome characterized by excessive dilatation of the urinary bladder without true outlet obstruction, hypoperistaltism and malrotation of intestines, and a very poor prognosis. It was proposed that neuropathic and myopathic disorders underlie the functional obstruction and dilatation of the bladder while the syndrome exhibits heterogenous histopathological features. Our case was a male fetus diagnosed by autopsy after intrauterine death, with typical findings of the syndrome. A discussion of the gross features in autopsy and histopathological-pathophysiological correlation is presented.