INTRODUCTION AND IMPORTANCE: Inflammatory myofibroblastic tumor (IMT) is an extremely rare sarcoma mainly reported in the lung. The adrenal gland, however, is an extremely rare site of occurrence. Herein, we report a case of a large adrenal mass with no contributory imaging features presenting a challenging pathological diagnosis. CASE PRESENTATION: A healthy 32-year-old male patient, with no personal nor family history, presented with loss of body weight and condition developing for two months. Physical examination revealed no abnormalities with a soft and depressible abdomen and absence of palpable lump. Circulating Chromogranin A and catecholamine metabolites were within normal range. The preoperative imaging features were noncontributory. The pathological examination on biopsy specimen concluded to a spindle cell proliferation set in a chronically inflamed background suggesting the diagnosis of IMT. The patient, subsequently, underwent radical nephrectomy unveiling a 25 cm adrenal mass. Pathological findings on resection specimen confirmed the diagnosis of IMT of the left adrenal gland. CLINICAL DISCUSSION: Due to their rarity in the adrenal gland and their noncontributory radiological features, IMTs are typically not considered as a potential diagnosis in this location. As a result, this condition is often underdiagnosed. In this setting, suspicion for IMT should be raised when spindle cell histology is observed. CONCLUSION: It is fundamental for practicians to keep this entity in mind as the treatment consists of complete tumor extirpation. The latter being the only guarantee of recurrence free follow up.