The present invention provides compositions of recombinant human lysosomalacid lipasehaving particular glycosylation patterns for internalization into targetcells, a vectorcontaining the nucleic acid encoding human lysosomal acid lipase, a host celltransformedwith the vector, pharmaceutical compositions comprising the recombinant humanlysosomal acid lipase and method of treating conditions associated withlysosomal acidlipase deficiency, such as Wolman disease.