Niemann Pick disease (NPD) is an inherited disorder that is due to defects in an enzyme called acid sphingomyelinase. This condition leads to abnormal accumulation of lipids in spleen, liver, lungs, bone marrow, and brain. NPD type A is known as the neurological type, which appears during infancy, with intellectual disability and death in early childhood. A less severe type of the disease, NPD type B is known as the non-neurological type because the neurological system is usually not affected. Children with this condition usually survive into adulthood. At this time, there is no effective treatment for NPD type A. Pathogenic variants in the gene SMPD1 can cause either type A or type B of NPD.