A patient with features of congenital βlipoprotein deficiency and intestinal lymphangiectasia is reported. The patient's illness was characterized by chronic diarrhea, mild steatorrhea, mild intestinal protein loss, and severe proximal muscle weakness. Plasma βlipoprotein levels were markedly decreased, and total cholesterol ranged from 84 to 98 mg/100 cc. An abdominal lymph node was infiltrated with lipophages. Intestinal biopsy showed clubshaped villi filled with PAS-negative macrophages and dilated lacteals. Electron microscopy showed lipid retention within intestinal absorptive cells, chylomicron retention, and numerous lipophages throughout the lamina propria. It is postulated that lipophage infiltration of abdominal lymph nodes resulted in the lymphangiectasia and protein-losing enteropathy. The malabsorption is presumably secondary to both lymphangiectasia and βlipoprotein deficiency.