Purpose <p>The objective of this case report is to present an atypical case of paraneoplastic immune-mediated myositis in a patient with recurrent clear cell carcinoma of the ovary.</p> Methods <p>Data on the patient’s clinical course, laboratory values and imaging were collected through chart review on the hospitals electronic medical record system.</p> Results <p>A 70-year-old female, with recurrent stage IC3 clear cell carcinoma of the ovary with three pericapsular cystic lesions in the right hepatic lobepresented to clinic for planning of second-line chemotherapy. However, her treatment course was delayed due to the uncommon development of paraneoplastic myositis causing her to be too medically unstable to initiate therapy. This contrasted with the more common scenario where myositis precedes cancer diagnosis and improves with tumour removal.</p> Conclusion <p>We recommend not only for evaluating malignancy in patients presenting with new onset myositis but considering paraneoplastic myositis in patients with ovarian cancer who present with suggestive laboratory markers even without the full clinical syndrome.</p>