Cardiac amyloidosis (CA) is caused by extracellular deposition of misfolded protein fragments in the myocardium, chiefly due to plasma cell dysregulation (AL amyloidosis) or transthyretin (ATTR amyloidosis). Amyloid deposition leads to irreversible damage to the myocardium, causing diastolic dysfunction and restrictive cardiomyopathy. Progression of the disease can lead to advanced restrictive disease and progress to severe systolic dysfunction. Treatment should primarily focus on addressing the fundamental pathology, targeting the underlying disease responsible for the amyloid deposition. Efficient disease-specific therapies are available for delaying the progression of the disease by reducing the accumulation of abnormal proteins. As amyloid deposition is largely irreversible, timely diagnosis and specific preventive therapy initiation at the earliest stages of disease progression are imperative. The main secondary goal is alleviation of clinical consequences of the failing myocardium leading to clinical heart failure (HF). The combination of restrictive physiology with increased filling pressures causes fluid accumulation and the restricted, fixed stroke volume as well as low heart rate leads to low cardiac output. Patients develop autonomic neuropathy with blunted sympathetic compensatory response. This makes regulation of volume status quite challenging and neurohormonal blockage not well tolerated. Alleviation of congestion includes salt restriction and judicial usage of loop diuretics and aldosterone antagonists with close monitoring of volume status. Neurohormonal blockage of the renin-angiotensin axis is contraindicated. Amyloid deposition in the atrium leads to significant atrial pathology and patients have a high risk of developing atrial fibrillation and embolic events. Treatment of rate or rhythm control is best managed with amiodarone. Beta blockers can be challenging but can be used in specific cases. Digoxin may be considered with low-dose and careful monitoring. Midodrine is very effective to increase blood pressure in patients that develop autonomic neuropathy. Syncope and sudden death due to conduction disease or pulseless activity can occur. Careful consideration to this should be given before initiating medications that effect the conduction system. Pacemaker implantation is indicated in appropriate cases and a defibrillator is indicated for secondary prevention of ventricular arrythmias. Patients often progress from restrictive cardiomyopathy to systolic dysfunction and develop advanced HF. Management of these patients is extremely challenging. Patients often develop renal failure and low cardiac output state with multi-organ failure and cardiogenic shock. Therapeutic options are very limited and prognosis is poor. Heart transplantation is an option in very select appropriate patients if extracardiac involvement is limited. Palliative therapy with alleviation of dyspnea and pain, should be offered.