Systemic amyloidosis is caused by the deposition of amyloid proteins in varying organ systems throughout the body, leading to dysfunction within those systems. The development of cardiac amyloidosis is one of the main indicators of poor prognosis in patients. Cardiac amyloidosis is most commonly caused by the immunoglobulin light chain amyloidosis and the transthyretin amyloidosis. Both have poor prognoses when associated with cardiac amyloidosis; however, the patients with the former subtype fair far worse than those with the latter. Despite amyloidosis having a history of being underdiagnosed, recent epidemiological data indicate that the rate of diagnosis has increased, which has coincided with improved in-patient median survival rates. It is of great importance that patients are diagnosed with the correct subtype as the main treatment strategy is to treat the underlying cause of amyloidosis. If a misdiagnosis is made, patients can receive treatment that might be ineffective or even harmful. A great progress has been made in pharmacological treatments for treating the underlying causes; however, many of the proposed treatments still need more evidence to support their use.