Selective fascicular involvement of the median nerve trunk above the elbow leading to anterior interosseous nerve (AIN) syndrome is a rare form of peripheral neuropathy. This condition has recently garnered increased attention within the medical community owing to advancements in imaging techniques and a growing number of reported cases. In this article, we explore the topographical anatomy of the median nerve trunk and the clinical features associated with AIN palsy. Our focus extends to unique manifestations captured through MRI and ultrasonography (US) studies, highlighting noteworthy findings, such as nerve fascicle swelling, incomplete constrictions, hourglass-like constrictions, and torsions, particularly in the posterior/ posteromedial region of the median nerve. Surgical observations have further enhanced the understanding of this complex neuropathic condition. High-resolution MRI not only reveals denervation changes in the AIN and median nerve territories but also illuminates these alterations without the presence of compressing structures. The pivotal roles of high-resolution MRI and US in diagnosing this condition and guiding the formulation of an optimal treatment strategy are emphasized.