A histological study of surgical specimens of idiopathic hydronephrosis demonstrated an abnormal muscle arrangement at the ureteropelvic junction in 18 of 26 cases. We believe this abnormality to be the cause in these cases. At this junction, the muscle bundles, instead of displaying the normal interwoven (braided) pattern, are arranged into an outer circular and an inner longitudinal layer. This abnormal muscle arrangement can be attributed to local failure of the physiological uncoiling during growth and development.