INTRODUCTION: Malakoplakia is a rare chronic inflammatory disorder, predominantly affecting the urinary tract. In the head and neck region, it is very rare and may confuse the clinicians during investigations, as features may mimic malignancy. MATERIALS AND METHODS: We report a case of malakoplakia involving the parotid gland and review of the reported cases of malakoplakia in head and neck region. RESULTS: Histologically, this is the first classic case report of malakoplakia involving the parotid gland in the world literature. A total of 49 cases have been reported in the head and neck region; 38.7% of these are cutaneous. In soft tissue, the tongue is the most common site. Salivary gland involvement is very rare. Previously, submandibular salivary gland involvement has been reported. CONCLUSION: A possibility of malakoplakia should be considered as a differential diagnosis in patients with enlarged head and neck masses. Histology is essential to diagnose this benign inflammatory disorder and to differentiate from a malignant process.
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