Burkholderia cepacia is an opportunistic Gram-negative bacillus that is found naturally in soil and water and usually causes respiratory infections in patients with cystic pulmonary fibrosis. Few cases of urinary tract infections with B. cepacia have been described in the literature, all of them clinical case presentations or case series. Therefore, we have compiled the data from the literature on this topic in a review to gain a better understanding of the etiopathogenesis, diagnosis and treatment methods of this disease. B. cepacia can lead to multidrug-resistant urinary tract infections in hospitals when surfaces and medical equipment are contaminated. The diagnosis is made after the onset of postoperative febrile syndrome or prolonged hospitalization in the intensive care unit. The evolution can be unfavorable, with the occurrence of sepsis and increased mortality.