BackgroundHyalinizing clear cell carcinoma (HCCC) is a rare, typically low‐grade malignant salivary gland tumor. We present a case of HCCC with multiple metastasis.Case PresentationThe patient was a 34‐year‐old woman with a tumor at the base of tongue. HCCC was diagnosed by FISH analysis for EWSR1‐ATF1 rearrangement after surgery. Systematic metastasis was identified at a three‐year follow‐up. Signs of Rouviere lymph metastasis and distant metastases (i.e., pleura, mediastinum, and gluteus maximus) were identified. The patient died because her condition deteriorated with multiple metastases.ConclusionThe potential for aggressive HCCC should be considered while offering treatment.