Malignancy can be accompanied by rheumatologic clinical findings, and these can occur prior to, concurrently with, or after a diagnosis of cancer. Certain clinical information helps to differentiate paraneoplastic symptoms from those more characteristic of classic rheumatologic disorders such as rheumatoid arthritis or systemic lupus erythematosus. Clinicians need to have a higher level of suspicion for malignancy in a patient with rheumatologic manifestations in certain populations. This may be the case in patients with a personal or family history of malignancy, atypical presentations of rheumatologic disorders, and certain clinical situations (such as hypertrophic osteoarthropathy, carcinoma polyarthritis, dermatomyositis, and unilateral sacroiliitis). The current standard of care for patients with a rheumatologic disorder with a known paraneoplastic association (i.e., dermatomyositis) is age-appropriate cancer screening. This review is designed to guide the physician to musculoskeletal signs and symptoms that may be paraneoplastic in origin. Additionally, when the clinician understands current information regarding malignant associations, it will help them decide when it may be appropriate to pursue further investigative work-up or evaluate for recurrence of a known primary tumor.