Prostate lymphoma is a rare and uncommon entity, accounting for only 0.09% of prostate tumors and 0.1% of all newly diagnosed lymphomas and an even rarer occurrence in young adults. The prostate, being an extralymphatic site, is infrequently involved in lymphoma, particularly in individuals without a history of immunosuppression or systemic disease.

A 26-year-old male presented with Gross painless intermittent hematuria with no associated clots. Patient sought consult with Urologist and on Clinical examination revealed no palpable abnormalities, with no signs of infection on Urinalysis and on Whole Abdominal CT Scan with IV contrast, with no noted significant findings on the prostate with no enlarged lymph nodes.

Patient underwent Cystoscopy was revealing Prostatic urethra with friable and hyperemic mucosa with sloughed off tissue, with areas of pinpoint bleeding. Biopsy of the area done showing Atypical Lymphoid Proliferation, immunohistochemical staining with CD20, OCT2, PAX5, CD179a, CD3, and KI-67 was done showing. DIFFUSE LARGE B-CELL LYMPHOMA with a non-germinal center B-cell. The patient currently undergoing chemotherapy (R-CHOP regimen) and is currently status post 3rd cycle. His clinical condition improved significantly post chemotherapy

Prostate lymphoma in a young adult is an extremely rare condition that should be considered in the differential diagnosis of patients presenting with unexplained hematuria. Early diagnosis and prompt treatment with chemotherapy offer a favorable outcome, highlighting the importance of timely intervention in such rare cases. This case underscores the need for awareness among clinicians regarding the possibility of prostate lymphoma in younger individuals without predisposing factors.