Primary bladder neck dysfunction is a non-neurogenic voiding disorder commonly diagnosed in young and middle age adult men, but frequently overlooked in children. Because these children typically present with lower urinary tract symptoms that also are associated with other more common forms of dysfunctional voiding, the diagnosis may be missed and treated with a variety of empiric modalities that ultimately fail. Although its underlying pathogenesis remains debated, the hallmark of the diagnosis is a failure of the bladder neck to properly open and allow for unimpeded urine flow. Videourodynamic evaluation is the only diagnostic tool that can urodynamically document the obstructive pressure/flow parameters of the condition while simultaneously localizing the functional obstruction to the bladder neck fluoroscopically. This article reviews the clinical and urodynamic findings of this entity in children and adolescents and our experience with a adrenergic antagonists, the current approach in medical therapy.