Primary EAC neoplasms include benign and malignant lesions of bony, glandular or cutaneous origin. Small, benign slow growing bony neoplasms are often asymptomatic, diagnosed incidentally and might not require intervention. Both malignant and benign neoplasms of cutaneous and glandular origin can present with symptoms of chronic otitis externa, leading to delays in diagnosis. Prompt biopsy of soft tissue lesions associated with non-resolving otitis externa are warranted. Local and regional imaging is helpful to understand disease extent and origin, but even early-stage malignant neoplasms require aggressive surgical treatment.