Two patients were carrying missense variants in the ABCC9/Sur2 gene with acromegalic features and hypertrichosis, but the normal growth hormone (GH) axis also had clinically non-functioning pituitary macroadenomas, a feature which has not previously been associated with C.S. C.S. is associated with gain-of-function (G.O.F) mutations of ABCC9 and KCNJ8 genes. The gene discussed is GH1; the disease is hypertrichosis.