AbstractObjectives/Hypothesis:Salivary gland obstruction caused by vascular malformation with phleboliths is relatively uncommon and may be masquerading as sialolithiasis. This article presents the case of a patient who suffered from vascular malformation with phleboliths that caused submandibular gland obstruction and was once misdiagnosed as sialolithiasis.Study Design:Illustrative case report and review of the literature.Methods:Our patient was presented with recurrent episodes of right submandibular swelling and pain at mealtime for two years. The radiopacities in the X‐ray film were misdiagnosed as multiple sialoliths. The sialendoscopic surgery ruled out the sialoliths, and an impressively expanded vascular network consisting of capillaries was detected all over the ductal lumen. We diagnosed vascular malformation with phleboliths. A search of the MEDLINE database (from 1948 to February 2009) was performed.Results:The patient was treated with sclerotherapy. The clinical outcome was satisfactory during a follow‐up of 28 months, with no evidence of recurrence. The results of the extensive literature review showed that vascular malformation with phleboliths that leads to submandibular gland obstruction is rare with a total of three cases reported, including this patient.Conclusions:Vascular malformation with phleboliths must be included in the differential diagnosis of salivary gland obstruction. Laryngoscope, 2009