 Wolman disease is caused by mutation of the lysosomal acid lipase LIPA gene. It is inherited as an autosomal recessive trait. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. The LIPA gene contains instructions for producing the enzyme lysosomal lipase acid. This enzyme is essential for metabolizing certain fats in the body, especially cholesterol specifically cholesteryl esters and to lesser degree triglycerides. Without proper levels of this enzyme, these fats abnormally accumulate in and damage various tissues and organs of the body. Mutations of the LIPA gene result in the lack of production of the LIPA enzyme or production of a defective, inactive form of the LIPA enzyme. Wolman disease is an extremely rare disorder that affects males and females in equal numbers. More than 50 cases have been reported in the medical literature. However, cases may go undiagnosed or misdiagnosed making it difficult to determine the disorders true frequency in the general population. Wolman disease is named after one of the physicians who first identified the disorder in the medical literature in 1956. 