population over 40 years old. Only ve cases accounted for in children below 14years old. There is no sex predilection of this disease. The most common location of IMT is the lungs; extrapulmonary sites include the head, neck, liver, retroperito­neum, ventricles, bladder, and spinal canal [2–10]. In the genitourinary system, the most common site is the bladder [11]. The kidney is an unusual site for IMT.Fifty percent of cases involve the left kidney and the remaining 50% of the cases in the right kidney. IMT was notably found to be more commonly occurring in the upper pole of the kidney than in the lower pole. The literature suggests that spindle cell proliferation is reactive hyperplasia that occurs due to chronic inammation, surgery, or trauma. However, etiological factors are still unknown. Long- standing nephroli­thiasis is also suspected as one of the causes of IMT.IMT was initially thought to be an inammatory pseudotumor. However, later research classies this as a borderline neoplasm because it was found that spindle cells were the main part of these tumors [1]. As per a few scholars, IMT has been redened as low-grade neoplasm. It is cru­cial to identify these tumors and treat them as it has a potential for metastasis, is locally invasive, and can recur. It is challenging to diagnose it preoperatively because it is a rare entity involving the kidneys and is often missed by the physician and mis­diagnosed as a malignant disease both clinically and pathologically [4, 5]. It occurs equally in both men and women. It can occur in a wide range of age groups. It is more often diagnosed in children than adults of both sexes equally. However, extra­pulmonary forms occur more commonly in adult women [2]. Most commonly it clinically presents mainly as lower back pain and abdominal pain. Other clinical presentations include hematuria, anemia, recurrent high fever, abdominal discom­fort, lethargy, and weight loss. It can also be an incidental nding in imaging. The tumor size reviewed ranged from 1.5cm to 13.5cm. In clinical practice, the rst-line treatment is surgical resection of the lesion. In patients with bilateral masses, single kidney, or renal insufciency, a biopsy with intraoperative pathological assessment was highly suggested by some authors to avoid needless removal. However, it is still disputable as the diagnosis can be made only post nephrectomy [22]. It is linked to the anaplastic lymphoma kinase (ALK) gene, located on 2p53 in 50% of cases. Fusion partners are clathrin heavy chain, tropomyosin, and ran-binding protein, correction-genes that have been seen in patients who are ALK-positive. Fusion pro­teins involved with ALK-negative genes are reactive oxygen species (ROS-1) and platelet-derived growth factor (PDGF) genes [12, 13]. ALK-positive cases are more commonly found in children. Herein, we discuss a case of renal IMT in a patient with a history of long-standing nephrolithiasis to improve the understanding of the disease and impart the patients with better treatment and management.