Cystic Lymphangioma (CL) is a rare condition that is typically observed as a congenital malformation in the head and neck. These congenital lesions usually appear before the age of two. Adult occurrences are extremely unusual, and only a few cases have been mentioned in the literature. CL is characterised by the collection of an epithelial lining of lymphatic fluid, which can be caused by lymphatic abnormalities, trauma, or surgery. In the present case report, the authors present the findings of a 49-year-old adult female who had painless, gradually increasing left-sided neck swelling for three years, with no other relevant medical history. The initial ultrasound revealed a large cystic lesion extending from the left sternocleidomastoid muscle to the left submandibular gland. The lesion measured 8×7×3 cm and exhibited narrow septae and loculation at the periphery. Avascularity was noted within the cyst wall and interlobular septae. The diagnosis favoured macrocytic lymphangioma/Congenital Lymphangioma (CH). During the surgical procedure, the cyst was dissected, and it was found to be located over the internal jugular vein, carotid artery, and spinal accessory nerve. The cyst was completely removed, and a surgical drain was left in place. The wound was sutured in two layers, and a sterile dressing was applied. The drain was removed on the third postoperative day, and the dressing with sutures was removed on the seventh postoperative day, revealing a healthy wound. The diagnosis was confirmed by histopathological examination {Haematoxylin and Eosin (H&E)} of the tissue specimen, which showed flattened epithelium lining the inside of the cyst. The cyst contained dilated gaps in a fibrocollagenous stroma infiltrated by lymphocytes. Therefore, CH can be considered as a differential diagnosis for head and neck masses in adults.
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