To study the potential role of sympathetic activity in the pathogenesis of arterial hypertension associated with autosomal dominant polycystic kidney disease (ADPKD) and to analyze its relationship with 24-hour blood pressure pattern, plasma catecholamines and 24-hour ambulatory blood pressure monitoring were evaluated in 30 ADPKD hypertensive patients (of which 17 without and 13 with renal failure) and in 50 essential hypertensives. The groups were matched for sex, body mass index, known duration of hypertension, and clinic blood pressure. Plasma catecholamines, determined in resting position, were higher in ADPKD patients without renal failure than in essential hypertensives. Nighttime diastolic blood pressure was higher and the percentage day-night difference in mean blood pressure was lower in hypertensives with ADPKD compared to patients with essential hypertension. Blood pressure was significantly correlated with plasma noradrenaline in ADPKD patients, independently of renal function. No significant differences were observed between ADPKD patients with and without renal failure, with respect to plasma catecholamines, 24-hour daytime and nighttime ambulatory blood pressures and the percentage day-night difference in mean blood pressure.