The Carney complex is an autosomal dominant syndrome characterised by multiple neoplasies, including myxomas at various sites (cardiac, cutaneous, mammary), spotty pigmentation, endocrine overactivity (Cushing's syndrome, acromegalia), testicular tumours and schwannomas. This report describes the case of a 43-year-old woman with an acute inferior myocardial infarction, probably due to coronary embolization from a large left atrial myxoma and who presented this association. A brief review of the Carney complex is provided after discussion of this rare case that required an screening of family members.