Chylothorax and chylous ascites with tuberculosis is a very rare condition in children and it refers to the accumulation of lipid rich lymph in the pleural and peritoneal cavities due to disruption of the lymphatic system. To date only two cases are reported in the literature in children aged <18 years. Here we are reporting a case of a 4-year-old boy who has Chylothorax and chylous ascites due to peritoneal tuberculosis which is complicated by medullary nephrocalcinosis and improved after antitubercular therapy.