Wolman disease and Cholesterol ester storage disease are autosoma lrecessive hereditary diseases characterized by a deficiency of the enzyme lysosomal acid lipase. Lysosomal acid lipase is a hydrolase which is essential for degradation of plasma lipoproteins in the lysosomes. Because of the inherent lack of lysosomal acid lipase leads to accumulation of cholesterol esters and triglycerides in tissues that most participate in mediated endocytosis receptors and these are the cells of the liver, spleen, adrenal glands, lymphnodes and cells in general monocyte macrophage system. Recent clinical studies show encouraging treatment by recombinant replacement therapy with sebelipaza alpha. Objective of this paper is to introduce screening methods for the measurement of lysosomal acid lipase in dried blood filter paper samples. By measuring total lipase activity and lipase activity in the presence of Lalistat 2, lysosomal acid lipase can be determined in dried blood samples. Results obtained by measurement after establishment of optimal conditions show good agreement with the literature data. The results of our „in house“ screening method have shown that the method can be used to distinguish healthy individuals from patients with a lack of lysosomal acid lipase. Method is not reliable for distinguishing heterozygotes from healthy individuals. Validation of the „in house“ method according to the appropriate protocol for dried blood filter paper samples is necessary when it is used in clinical laboratories as a method in suspected lack lysosome acid lipase.Validated method of selecting from dried blood filter paper samples will enable greater availability of diagnosing a missing lysosomal acid lipase and the timely introduction of enzyme replacement therapy for so far incurable hereditary metabolic disorders (Wolman disease and Cholesterol ester storage disease).