Background Amyloidosis is an infiltrative cardiomyopathy caused by amyloid deposition into the myocardium. In recent years, recognition of this treatable cause of heart failure has increased. There are striking sex differences in diagnosis, clinical course, and outcome. Notably, women have a worse prognosis than men with similar amounts of cardiac involvement. Methods This review provides an overview of the current state of knowledge on the epidemiology, clinical features, diagnosis and treatment of amyloid heart disease. The differences observed between men and women are discussed and recent advances in the field are highlighted. Results Compared with men, women are generally older at diagnosis, appear to have less severe cardiac disease at time of impairment and are given a late diagnosis. The less apparent disease in women may be responsible for the delay in diagnosis. Women may be under-diagnosed using neutral sex diagnostic criteria. Conclusions Addressing diagnostic disparities may require the use of sex-specific diagnostic thresholds, as well as a more expansive use of multimodality imaging. Future studies trials should aim to enroll greater numbers of female participants to inform optimal therapeutic approaches and to define the sex-specific disease phenotype for this increasingly treatable disease.