In this article recent data on cardiac amyloidosis diagnosis, assessment of the severity and prognosis, as well as contemporary treatment methods are reviewed. A clinical case of primary cardiac amyloidosis (AL) which appeared in middle-aged woman with rapidly progressing clinical symptoms of heart failure and syncope, is presented. Suspicion of cardiac amyloidosis should arise in case of restrictive cardiomyopathy along with significant thickening of heart walls and other organs damage. Significant thickening of the left ventricle is not associated with ECG signs of myocardial hypertrophy, pseudo-infarction pattern may be found in the precordial leads (V1–V3) without previous myocardial infarction. For patients with amyloid deposition confirmed with non-cardiac biopsy, involvement of heart can be confirmed by positive heart biopsy and/or left ventricular wall thickening (interventricular septum > 12 mm) in the absence of hypertension or other potential causes of left ventricular hypertrophy. Contemporary methods of amyloid visualization, biochemical markers and quantitative methods for detection of amyloid proteins allow earlier and more accurate diagnosis of the disease.