Renal tubular dysgenesis, a rare, lethal, autosomal recessive disorder
, is characterized by short and poorly differentiated proximal convolu
ted tubules associated with oligohydramnios, Potter sequence. and neon
atal death due to respiratory failure. Abnormalities of the skull may
occur in some cases. We report an infant born of a consanguineous unio
n who also had microcephaly, among other features, A history of oligoh
ydramnios with or without skull abnormalities and a lethal outcome wit
hout obvious explanation should occasion renal histological study aime
d at precise diagnosis and genetic counselling.