PT - JOURNAL ARTICLE AU - Vishal Jha AU - Aditi Jha TI - Unusual case of chylothorax with unilateral limb swelling AID - 10.1136/bcr-2019-230379 DP - 2020 Feb 01 TA - BMJ Case Reports PG - e230379 VI - 13 IP - 2 4099 - http://casereports.bmj.com/content/13/2/e230379.short 4100 - http://casereports.bmj.com/content/13/2/e230379.full SO - BMJ Case Reports2020 Feb 01; 13 AB - Here, we present an unusual case of 26-month male toddler who presented with swelling of right lower limb with painless hyperpigmented patch over right groin of 18 months duration associated with recent onset respiratory distress. Evaluation revealed right chylothorax and MRI revealed altered signal intensity in bones and muscles of right lower limb. Lymphoscintigraphy revealed absence of lymphatic channels in right lower limb. Skin biopsy from hyperpigmented patch was suggestive of vasoformative lesion favouring lymphangiomatosis. A diagnosis of Gorham’s syndrome was made, and our patient was managed with drainage of chylothorax followed by pleurodesis, parenteral nutrition and radiotherapy.