Objective To investigate the methods of medical nutrition therapy and combined therapy for patients with primary intestinal lymphangiectasia (PIL). Methods Six PIL patients with malnutrition were enrolled. Medical nutrition therapy and other therapy methods were given. Results There was a trend that the patients' nutritional statuses were improved after medical nutrition therapy with medium-chain triglyceride diet, which was based on enough energy,high protein,low fat,low dietary fiber, and low salt. During their hospitalization, their total body weight increased by (4.5±1.9)kg, serum albumin increased from (19.8±2.9)g/L to (32.7±2.2) g/L (P=0.056),and serum total protein increased from (41.5±5.9)g/L to (56.0±7.1) g/L (P=0.08), and prealbumin increased from (189.7±24.7)g/L to (275.3±42.8) mg/L (P=0.26). All the patients were discharged from the hospital eventually. Conclusion The combined therapy including medical nutrition therapy with medium-chain triglyceride diet may improve the nutritional status and promote the recovery process of patients with PIL.