Niemann Pick disease (NPD), Described by Niemann and Peak in 1914 and 1922 respectively, it is a relatively rare disease caused by the disorder of genetic phospholipid metabolism, which is an autosomal recessive inheritance. One third of the patients with this disease have a family history, mainly due to the abnormal deposition of sphingomyelinase in the liver, spleen and other parts of the body to form Niemann peak cells, which causes the liver, spleen and other system diseases. The pathological diagnosis of this case is type B and the prognosis is good.