The term congenital ‘bladder outlet obstruction (BOO)’ describes the collection of conditions in which the normal, urethral egress of urine from the fetal bladder is impaired. The term is interchangeable with fetal ‘lower urinary tract obstruction’, as used by other authors. After considering normal urinary tract embryology, we describe the epidemiology of congenital BOO and the primary anatomical disorders associated with it. We then proceed to describe its fetal and postnatal clinical manifestations and then consider therapies and interventions which have been used to manage the condition. We not only focus on urethral and bladder disease with constitutes BOO itself, but also describe associated kidney disorders which, via chronic renal excretory failure, are important causes of morbidity. Rather than provide an exhaustive review, we emphasise studies published in the last decade, and therefore readers are referred to other reviews citing numerous earlier references.