Background: Syringocystadenoma papilliferum represents an uncommon benign skin appendage hamartoma, typically manifesting in neonates or early childhood. The concurrent presentation of this lesion with parotid gland malignancy has not been previously documented in the medical literature. Case Presentation: A 19-year-old female presented with a progressive exophytic papillated mass in the left infrauricular region spanning 12 months, with recent serous drainage. Clinical examination revealed a firm, fixed nodule measuring 3×2 cm with characteristic serosanguineous discharge. Imaging demonstrated a heterogeneous soft tissue density involving the superficial parotid lobe and extending to cutaneous margins. Initial fine needle aspiration cytology suggested granular cell tumor; however, punch biopsy and immunohistochemical analysis (CK19 positive) established syringocystadenoma papilliferum. Superficial parotidectomy with wide local excision was performed. Final histopathological examination revealed coexistent acinic cell carcinoma, representing the first documented case of this association. Clinical Significance: This case illustrates the diagnostic challenges in evaluating complex parotid masses and the importance of comprehensive histopathological examination. The coexistence of a benign skin hamartoma with primary parotid malignancy expands the differential diagnosis spectrum for infrauricular masses. Outcome: The patient achieved complete surgical excision with clear margins and remained disease-free at 8-month follow-up with excellent cosmetic outcomes and preserved facial nerve function.