NT-pro-brain natriuretic peptide (NT-proBNP) values are correlated with right ventricle dysfunction in pulmonary hypertension and have been recommended as a prognostic marker for symptomatic primary pulmonary hypertension. The purpose of this pilot study was to determine whether NT-proBNP values correlate with mild to moderate pulmonary hypertension in Gaucher disease. The NT-proBNP assay was performed on sera from patients with type I (non-neuronopathic) Gaucher disease who were known to have tricuspid insufficiency (TI) gradient values on echocardiography that were normal (TI<20mmHg), borderline (TI=25–29mmHg), or abnormal (TI≥30mmHg) over time. There was a statistically significant correlation (p=0.05) between mean NT-proBNP values and TI gradient group: mean NT-proBNP for normal TI gradient (n=20 patients)=133.3 (range: 46–445)pg/ml; mean NT-proBNP for borderline TI gradient (n=17 patients)=288.7 (range: 81–1088)pg/ml; and mean NT-proBNP for elevated TI gradient (n=10 patients)=1034.2 (73–6703)pg/ml. Treatment status was not correlated with TI gradient or NT-proBNP values. Thus, in this pilot study of Gaucher disease that included patients with mild to moderate pulmonary hypertension, NT-proBNP was correlated with echocardiographic findings. Further studies will be required to ascertain if NT-proBNP can be used for diagnosis and monitoring of these patients.