Lysosomal storage diseases (LSDs) are inborn errors of metabolism, the hallmark of which is the accumulation, or storage, of macromolecules in the late endocytic system. They are monogenic disorders that occur at a collective frequency of 1 in 5000 live births and are caused by inherited defects in genes that mainly encode lysosomal proteins, most commonly lysosomal enzymes. There are over 60 LSDs, all of which are rare, and as a result, may go undiagnosed or misdiagnosed for some time. Gaucher disease (GD) is one of the most common LSDs. It has been shown that due to clinical features of hepatosplenomegaly, cytopenias, and bleeding diatheses, approximately 75% of patients will see a hematologist and up to three specialists prior to diagnosis. Although there has been increased awareness of the disease since the availability of treatment, there has been no significant decrease in the time to diagnosis. Some LSDs have associations with hematological malignancy. Despite their rarity, it is prudent that hematologists have an awareness of the various LSDs, since they may play a vital role in their diagnosis and management.