INTRODUCTION AND IMPORTANCE: Primary urethral carcinoma (PUC) is a rare cancer, comprising less than 1 % of all genitourinary malignancies, with a male predominance (3:1 ratio), and typically affects those over 75 years of age. PUC shows varied histological subtypes based on their location and sex. The prognosis depends on age, tumor grade, TNM stage, histology, and site. Organ-preserving surgery is for distal disease becoming a preferred option. CASE PRESENTATION: We report the surgical management of a male patient presenting with a history of urethral meatal ulcer, bloody urethral discharge, dysuria, and urine spraying, initially misdiagnosed as a persistent herpes ulcer. Examination revealed a 1 × 2 cm erythematous plaque over the urethral meatus. Pelvic magnetic resonance imaging (MRI) showed a 1.7 × 1.7 cm lesion in the distal penile urethra invading the corpus spongiosum, and a biopsy confirmed squamous carcinoma in situ. The patient underwent partial glansectomy and anterior urethrectomy, but pathology showed well-differentiated squamous cell carcinoma with positive margins. The patient was successfully treated with a total anterior urethrectomy, partial glansectomy, and augmented perineal urethrostomy. CLINICAL DISCUSSION: PUC (primary urethral carcinoma) presents nonspecifically, requiring high suspicion for diagnosis. Historically, treatment included total penectomy with cystoprostatectomy for proximal tumors and partial or radical penectomy for distal tumors. Distal tumors often have better outcomes, and organ preservation surgery is possible for selected patients, with no local recurrence in those treated with additional surgery or adjuvant radiation for positive margins. CONCLUSIONS: PUC is a rare urological malignancy that is challenging to diagnose and treat. Clinical stage and tumor location are critical prognostic factors for urethral carcinoma in men. Organ-preserving surgery is the preferred treatment for distal disease, with a 5-year overall survival rate of approximately 50 %.