BACKGROUND: Brucellosis is one of the most prevalent zoonotic diseases worldwide, posing a significant public health concern across both developed and developing nations. While musculoskeletal system involvement is the most common form of focal brucellosis, the genitourinary system represents the second most affected site. However, Brucella-related kidney involvement remains a rare manifestation of genitourinary brucellosis. CASE PRESENTATION: We herein present the case of a 55-year-old Iranian male patient, initially admitted with symptoms indicative of osteoarticular brucellosis, who subsequently developed pyelonephritis confirmed by positive urine culture for Brucella. He was treated with gentamicin for 10 days and rifampin and doxycycline for a period of 6 weeks. The patient fully recovered. CONCLUSION: Our case highlights the importance of considering brucellosis as a potential cause of pyelonephritis, especially where brucellosis is endemic and when musculoskeletal involvement is present. Additionally, a comprehensive review of the literature on kidney involvement in brucellosis was conducted to provide further insights into this condition. Clinical Trial number Not applicable.