<p>Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the bladder is uncommon and is often confused clinically and radiologically with urothelial carcinoma of the bladder. We present a rare case of a 46-year-old gentleman who presented with painless gross hematuria for 5 months. The patient had a past history of staged post-traumatic urethral stricture repair 35 years back and was on regular self-dilation for poor stream. CT urography and Multiparameteric MRI pelvis revealed diffuse enhancing urinary bladder wall thickening, primarily of the right lateral and posterior walls, involving both the VUJ which was T2 hyperintense and T1 hypointense (VIRADS 4). Cystourethroscopy revealed a diffuse and irregularly thickened bladder wall primarily in the right lateral and posterior walls. Bilateral ureteric orifices were not visible. Transurethral biopsy was obtained from the right lateral wall and histopathology report suggested an extranodal marginal zone B cell lymphoma. The patient received 4 cycles of chemotherapy with Bendamustine and Rituximab and is doing well on 9 months of follow up. Primary extranodal marginal zone lymphoma of the urinary bladder is an uncommon lesion and its diagnostic features may not be well known to the unaccustomed practitioner. Potential misdiagnosis of poorly differentiated urothelial carcinoma or chronic cystitis can occur clinically and radiologically. Accurate diagnosis depends upon comprehensive immunohistochemical and molecular workup on histopathology. It responds well to chemotherapy, radiation, or surgery. Limited literature exists on the presentation and treatment of this rare malignancy.</p>