The rare combination of intestinal lymphangiectasia with malrotation of the 
duodenum in a child of three months of life is described. Basing on the 
literature review only 3 similar cases were described in the world practice. The 
boy with protein-losing enteropathy was examined at Moscow Scientific Centre of 
Children's Health. The child had vomiting, diarrhea, loss in body weight, 
hypoproteinemia, lymphopenia. The infectious nature of the disease was excluded. 
It had been suggested the Waldman desease (primary intestinal lymphangiectasia). 
The prognosis for such disease is unfavorable. An examination of the child was 
continued against the backdrop of ongoing symptomatic therapy. Complete physical 
examination included monitoring laboratory blood tests, X-ray examination with 
contrast, CT-scan, gastroduodenoscopy with biopsy of the mucosa of the small 
intestine. Malrotation duodenum with the recurrent mid-gut volvulus with the 
development of secondary intestinal lymphangiectasia was diagnosed. Modern 
methods of examination and multidisciplinary approach made it possible to 
diagnose the case. Operation to eliminate fixation duodenum resulted in the 
recovery of the patient. At the present time the child grows and develops 
according to age and does not require treatment. The prognosis for this disease 
is regarded as favorable.