Myeloid sarcoma (MS) is a rare extramedullary neoplasm that can present in association with acute myeloid leukemia, most commonly in children younger than 15 years. This unique extramedullary malignancy may involve a variety of different organ systems and can present following, preceding, simultaneous with, or in insolation to acute myeloid leukemia. Common areas of extramedullary involvement include soft tissues, bones, lymph nodes, and the peritoneum. Imaging plays a critical role in the diagnosis and management of MS, with commonly used modalities including positron emission tomography-computed tomography, magnetic resonance imaging, computerized tomography, and ultrasound. The purpose of this review article is to provide radiologists with a comprehensive guide summarizing the relevant imaging and clinical features of MS, with emphasis on the role of imaging in the diagnosis, treatment, and follow-up of patients with MS. The relevant pathophysiology, epidemiology, clinical presentations, and differential diagnosis of MS will be reviewed. The relevance of different imaging modalities in diagnosis, monitoring of treatment response, and assessment of treatment-related complications will also be outlined. Through summarizing these topics, this review article aims to provide radiologists with a guide for understanding the existing knowledge of MS in the literature and the current role of imaging in the management of this unique malignancy.