Primary intestinal lymphangiectasia is a rare cause of protein-losing 
enteropathy and usually presents with intermittent diarrhea or malnutrition. 
Diagnosis depends largely on its pathologic condition demonstrating greatly 
dilated lymphatics mainly in the lamina propria of the mucosa. We report a case 
of primary intestinal lymphangiectasia, of the diffuse type, presenting with 
abdominal pain and voluminous diarrhea in a previously healthy 8-year-old boy. 
He had periumbilical pain for 3 months before presentation. He was managed by 
segmental bowel resections and end-to-end anastomoses. The histopathologic 
condition of the resected small intestine showed lymphatic dilation limited 
mainly to the subserosa and mesentery but was not prominent in the mucosa. 
Abdominal pain and diarrhea subsided postoperatively. The present case is the 
fourth report describing a response to operative resection.