Wolman disease is characterized by severe diarrhea and malnutrition leading to 
death during infancy. Lysosomal acid lipase deficiency is the cause of the 
symptoms and signs. It is inherited in an autosomal recessive manner. All Wolman 
disease patients have adrenal gland calcification. Previous therapeutic attempts 
have failed to provide remission. We report successful long-term bone marrow 
engraftment in a patient with Wolman disease resulting in continued 
normalization of peripheral leukocyte lysosomal acid lipase enzyme activity. 
Diarrhea is no longer present. Now, at 4 years of age, this patient is gaining 
developmental milestones. Cholesterol and triglyceride levels are normal. Liver 
function is normal. This is the first long-term continued remission reported for 
Wolman disease.