Disclosed is the use of a recombinant human lysosomal acid lipase (rhLAL) for the preparation of a medicament for treating a LAL deficiency in a human patient, wherein said medicament comprises rhLAL in an amount effective to reduce the serum or blood level of a liver transaminase to a normal level, wherein the medicament is formulated for administration between one time about every 7 days and one time about every 30 days and the administration is sufficient to reduce liver damage of said human patient.
