Introduction and Importance:To document a case of an intraosseous angiolipoma (IOAL) of the humerus.Case presentation:A 52-year-old woman presented with gradually increasing pain in her right arm. A X-ray series of the right elbow and computed tomography revealed a nonexpanding radiolucent lesion in the distal end of the right humerus. The fatty nature of the lesion was further verified using fat-suppression techniques on magnetic resonance imaging sequences.Clinical discussion:The tumor was surgically excised, and the patient has experienced no symptoms for 2 years now. Histopathological findings confirmed the presence of an IOAL, which is an extremely rare intraosseous tumor that consists of thin blood vessels and mature adipose tissue.Conclusion:Accurate diagnosis of an IOAL is challenging; therefore, careful planning and assessment are paramount in the management of such lesions, with histological findings being essential for a conclusive diagnosis and surgery is the most suitable treatment choice in most cases.