This study aimed to evaluate the clinicopathological features of mucocutaneous diseases with manifestation in the head and neck region. A retrospective analysis of a dermatology reference center database was carried out. Over 24 years. Clinicopathological data were collected from medical records and the data was analyzed by descriptive statistics. A total of 11.538 medical records were analyzed, being 152 cases of mucocutaneous diseases with manifestations in the head and neck region. Cutaneous lupus erythematosus was the most prevalent diagnosis (66.4%). Face (44.1%), females (79.6%), and patients with 45 years mean age were the most common features. In the oral cavity, the most affected region was the buccal mucosa (37.5%). Mucocutaneous diseases with head and neck manifestation were rare in the sample analyzed (1.3%), with cutaneous lupus erythematosus and lichen planus being the most common lesions in this region.