A 17-month-old baby had symptoms of malabsorption and partial intestinal obstruction. These were found to be caused by obliteration of the jejunum by solitary intestinal fibromatosis. Solitary intestinal fibromatosis, which may imitate malabsorptive diseases of the intestine, is a rare cause of intestinal obstruction in the neonatal period and infancy. Diagnostic aids and differential diagnosis of this rare disease are discussed, and previously reported cases are reviewed.