| Summary: | Introduction Lymphatic malformations are benign congenital lesions of abnormal lymphatic channel common in head and neck region. Extensive disease involving the larynx and presenting as a laryngeal pathology is a rare occurrence and can cause diagnostic enigma. Case report We report an unusual case of an extensive mixed cystic type of lymphatic malformation in the larynx without any external neck or oral swelling presenting in stridor and clinically mimicking as juvenile respiratory papillomatosis. Endoscopic debulking and tracheostomy were done and subsequent MRI showed lymphatic malformation. After the failure to inject doxycycline sclerotherapy properly, definite surgery was planned. However, the patient was lost to follow up. Discussion Lymphatic malformation extending into the larynx is a rare cause of airway problem. History and clinical examination alone is not always adequate in every case. In the case of an unusual presentation, the final diagnosis should include appropriate imaging and should be confirmed by histopathology.
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