Background: Phlebectasia is a term used for the dilatation of a vein without tortuosity. Rarely, the condition may affect the internal jugular vein (IJV), causing prominence in the neck, which may be confused with other neck swellings. Clinical Description: A 6-year-old boy presented with bilateral episodic swelling in the neck noticed since 2 years of age and a hoarse voice since 3 years of age. On examination, the neck swelling was soft, cystic, compressible, nontender, and nonpulsatile, increasing in size by all maneuvers increasing intrathoracic pressure. Systemic examination revealed the apex beat was on the right side, and the liver was palpable below the left subcostal margin. Management and Outcome: The workup for tuberculosis was negative, and direct laryngoscopy was normal. X-ray of the chest with abdomen showed dextrocardia and stomach shadow on the right side. Color Doppler revealed nontortuous dilatation of both IJVs. Dynamic computed tomography venography confirmed the diagnosis of bilateral IJV phlebectasia (IJVP). The child was started on speech therapy. The mother was counseled regarding the benign nature of the disease and advised regular follow-up. The child had no complications, and his voice improved with speech therapy at 1-year follow-up. Conclusion: Pediatricians should be aware that IJVP may rarely be the cause of neck swelling associated with hoarseness of voice. Increase in the size of the swelling with Valsalva maneuvers and color Doppler ultrasound helps to confirm the diagnosis.