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Primary intestinal lymphangiectasia (PIL) is a rare cause of lymphatic dysfunction leading to enteric protein loss in children. This case report describes a male child, with familial short stature who presented with chronic diarrhoea, anaemia, anasarca, lymphopaenia and hypoalbuminaemia due to PIL. Diagnosis was confirmed through a combination of endoscopic findings and histopathological analysis of small bowel biopsies showing dilated lymphatic vessels in the duodenum. The child responded to dietary intervention with medium chain triglycerides, fat soluble vitamin supplementations and subcutaneous octreotide therapy. The mainstay of management involves nutritional interventions with consideration of other supportive and therapeutic interventions.