Primary urethral carcinoma (PUC) is a rare cancer entity accounting for <1% of all genitourinary malignancies. Urothelial carcinoma is the predominant histological type of PUC (54–65%) followed by squamous cell carcinoma (16–22%) and adenocarcinoma (10–16%). Diagnosis of PUC depends on urethrocystoscopy with biopsy. Pathological staging and grading are based on the TNM classification and the 2016 WHO grading system. Local tumor extent and regional lymph nodes are assessed by magnetic resonance imaging and the presence of distant metastases by computed tomography. For all patients with localized distal tumors (≤T2N0M0), partial urethrectomy or urethra-sparing surgery is valid treatment options, provided negative intraoperative surgical margins can be achieved. Prostatic Ta-Tis-T1 PUC can be treated with repeat transurethral resection of the prostate and Bacillus Calmette-Guérin instillation therapy. In prostatic and proximal ≥T2N0 disease, neoadjuvant cisplatin-based chemotherapy should be considered prior to radical surgery. All patients with locally advanced disease (≥T3N0–2 M0) should be discussed within a multidisciplinary team. In patients with local urethral recurrence, salvage surgery or radiotherapy is a valid option. For patients with distant metastatic disease, systemic therapy can be offered based on the underlying histology.