BACKGROUND: Having a low nephron number is a well-known risk factor for hypertension. There is an inverse relationship between the filtration surface area and systemic hypertension. A significant percentage of masked hypertension can be detected in children with nephron loss by ambulatory blood pressure monitoring (ABPM). METHODS: We prospectively investigated ABPM results of children having reduced kidney mass with normal office blood pressures (BPs) and kidney function. Forty-three children with congenital solitary kidney (group 1), 11 children with acquired solitary kidney (group 2), and 76 children with hypofunctioning kidney (group 3) were compared with age, gender, and BMI-matched healthy control group (group 4). The dietary salt intake of 76 patients was evaluated as salt equivalent (g/day). The primary endpoint was change from baseline in mean 24-h ABPM variables and the proportion of patients with masked hypertension when assessed by ABPM. RESULTS: The masked hypertension ratio of all patients was 12.3% when assessed with ABPM. Night hypertension was significantly higher in all patient groups than in the control group (p = 0.01). Diastolic BP loads of groups 1 and 3 were higher than in controls (p = 0.024). Systolic BP loads were higher only in group 1 than in the control group (p = 0.003). The dietary salt equivalent of patients in group 1 correlated positively with 24-h SBP and mean arterial pressure (MAP) values. Patients with excessive dietary salt intake in group 1 had a significantly higher diastolic BP load than those without excessive salt intake in group 1 (p = 0.002). CONCLUSIONS: Masked hypertension can be seen in children with a solitary kidney or when one of the kidneys is hypofunctioning. Systolic BP loads are higher in children with congenital solitary kidney, and salt intake correlates with systolic BP profiles especially in those. Our results suggest that being born with a congenital solitary kidney increases predisposition to hypertension and salt sensitivity.