Mullerianosis is a sporadic condition of the urinary bladder. The study aims to present a case of mullerianosis with a brief literature review.A 52-year-old lady presented with hematuria for one-year duration. A general urine examination showed microscopic hematuria. Abdominal and transvaginal ultrasonography revealed a soft tissue mass (13 * 10 mm) within the base of the urinary bladder. Cystoscopy showed 1.0 * 1.3 cm benign-looking mass bulging into the posterior bladder wall covered by a normal bladder mucosa. Transurethral resection was done. The histopathological examination was consistent with mullerianosis.Two hypotheses have been proposed; the first one (implantation theory) believing that at the time of pelvic surgery and caesarian delivery Mullerian tissues become implanted inside the bladder. The second theory (metaplastic theory) holds the probability of differentiation of Mullerian epithelium to endometrial, endocervical, and tubal components and proliferation in the bladder.mullerianosis is a very infrequent entity that mainly affects the urinary bladder and is mainly reported in females during the reproductive period, medical and surgical treatment are both regarded as the management options.