Idiopathic orbital myositis is a rare inflammatory condition that predominantly affects multiple extraocular muscles. Isolated involvement of a single muscle is very uncommon. Isolated levator palpebrae myositis is a diagnostic challenge that should be considered in patients with periocular pain and complete eyelid ptosis without ophthalmoparesis or pupillary dysfunction. We propose a comprehensive set of diagnostic tests to identify the underlying etiology and a recommended treatment regimen. Additionally, we present a case from our clinical practice that illustrates this rare presentation, of which few cases have been described in the literature.