We describe the case of a 64-year-old patient with glucose-6-phosphate dehydrogenase deficiency who was referred to our hospital because of an acute inferior myocardial infarction.Given the possible risk of acute haemolytic anaemia, aspirin was not given in the acute phase, and the patient was successfully treated by balloon angioplasty of the right coronary artery.After functional and genetic testing showing the presence of the Mediterranean mutation, known to be a class II variant, the patient received oral daily aspirin (100 mg) under strict monitoring in order to promptly detect any sign of haemolysis. After 4 days, a complex percutaneous coronary intervention with an implantation of two drug-eluting stents was successfully performed on the left coronary artery. After 3 months, the patient is free from adverse events.Glucose-6-phosphate dehydrogenase deficiency is commonly considered a contraindication to aspirin intake; however, this case shows that aspirin at low, antiplatelet dosage is well tolerated and should not be denied to patients with ischaemic heart disease and complex coronary anatomy.