The Burkholderia cepacia complex (Bcc) comprises a group of genetically related yet phenotypically diverse Gram-negative opportunistic pathogens. These organisms demonstrate significant pathogenicity in immunocompromised populations, particularly cystic fibrosis (CF) and chronic granulomatous disease (CGD) patients, causing severe infections including pneumonia, bacteremia, and urinary tract infections. Notably, Bcc infection in CF patients may progress to acute respiratory failure or the life-threatening "cepacia syndrome". Of particular concern is the widespread nosocomial colonization by Bcc strains coupled with their sophisticated multidrug resistance mechanisms, which contribute substantially to clinical treatment failures. This review systematically examines the epidemiological characteristics, clinical manifestations and resistance mechanisms of Bcc. The review aims to provide evidence-based references for improving accurate diagnosis and enhancing infection control measures against this challenging pathogen.