Perivascular epithelioid cell tumor (PEComa) is exceptionally rare primary mesenchymal neoplasm occurring in the head and neck region. Based on literature review, we present here a first report of PEComa of the oropharynx morphologically masquerading as alveolar soft part sarcoma (ASPS). A 28-year-old woman presented with 1.5 weeks history of sore throat, hemoptysis, and dysphagia. Imaging (CT scan and PET-CT) of the neck revealed a 7.2 cm oropharyngeal mass with curvilinear calcifications with extension into the retropharyngeal/parapharyngeal space without lymphadenopathy or involvement of any other site. Microscopic examination of the oropharyngeal biopsies showed diffuse proliferation of tumor cells arranged in vaguely nested pattern associated with a delicate vascular stroma, thin fibrotic bands with interspersed lymphocytes, confluent necrosis, and scattered dystrophic calcifications. The tumor cells were large, polygonal to epithelioid, with eosinophilic granular and clear cytoplasm, centrally placed round to oval vesicular nuclei with occasional prominent nucleoli. Occasional mitotic figures were noted (up to 6 /10 hpf) including few atypical forms. Immunohistochemical panel showed tumor cells positive for HMB45 (focal), pan-melanoma cocktail (focal), SMA, TFE3 (diffuse), and desmin (scattered cells), and negative for various cytokeratins (including HMW and LMW), synaptophysin, S100, myogenin, MyoD1, inhibin, OCT-4, SALL-4, MITFD5, and SOX-10. Proliferation index as assessed by Ki67 was 20%-25%. FISH analysis was positive for TFE3 gene rearrangement and negative for TFE3/ASPSCR-1 rearrangement [(X;17) translocation]. Sarcoma gene fusion panel by next-generation sequencing did not detect any fusion transcript. The patient is currently being treated with everolimus therapy. This case highlights the rare presentation of malignant PEComa of the oropharynx, which should be considered as the differential diagnosis, especially the epithelioid variant of PEComa, which can mimic as ASPS, paraganglioma, or melanoma (when certain melanocytic markers are positive).