Melkersson-Rosenthal syndrome is an uncommon disorder which is presented by a triad of lip swelling, facial nerve palsy, and a fissured tongue. A number of treatments have been reported for this syndrome with variable outcomes. We have reported a 38-year-old female patient with lower lip swelling and a fissured tongue on clinical examination. She was also suffering from facial nerve paralysis since 8 years ago. A significant improvement of the signs was achieved with a combination of intralesional corticosteroid injection and oral azithromycin. Although our patient showed the triad, she had an atypical clinical course due to the initiation of syndrome with facial nerve palsy, resulting in a delay in definite diagnosis of her disease. The anti-inflammatory and immuno-modulatory effects of azithromycin and the therapeutic properties of the intralesional injection of corticosteroid have an important role in managing this syndrome.