To present a case of orbital granulomatous giant cell myositis and review the literature. We describe the case of a 51-year-old woman, with a past history of melanoma, who presented with an acutely painful orbital myositis. This evolved into a chronic relapsing process involving multiple muscles bilaterally; which demonstrated partial steroid responsiveness. Biopsy revealed a granulomatous giant cell myositis. Orbital granulomatous giant cell myositis is a rare histological entity which has an association with giant cell myocarditis and underlying malignancy.