OBJECTIVES: To describe a rare case of chemodectoma, its clinical features and management, and to discuss its relationship chronic hypoxia from Tetralogy of Fallot.

METHODS:
Design: Case Report

Setting: Tertiary Government Hospital

Patient: One

RESULTS: A 23-year-old woman presented with a painless, slow growing, movable right submandibular mass, initially diagnosed as a lipoma by fine needle aspiration biopsy.  Computed tomography scan showed a solid nodule with ill-defined margins from the angle of the mandible to the level of the hyoid bone along the carotid sheath. There was also an incidental finding of patent ductus arteriosus and Tetralogy of Fallot on pre-operative clearance. Excision of the mass under general anesthesia revealed adherence to the posterior portion of the carotid trunk enveloping both the internal & external carotid artery. Final histopathological diagnosis was chemodectoma.

CONCLUSION: Although rare, chemodectoma should be considered as one of the differentials in a patient with a submandibular mass. Hyperplastic chemodectoma may result from chronic hypoxia due to Tetralogy of Fallot.  Surgical excision is the treatment of choice.