The presence of macroamylasemia should be considered in the differential di
agnosis of hyperamilasemia associated with acute abdominal pain, with suspe
ct of pancreatitis. This uncommon and poorly known abnormality is defined a
s a blinding of serum amylase to certain proteins forming a circulating mac
rocomplex which prevents renal clearance of serum amylase. Two clinical cas
es as well as a review of this rare entity are here reported.