Bacillus cereus can cause serious, life-threatening, systemic infections in immunocompromised patients. The ability of microorganism to form biofilm on biomedical devices can be responsible for catheter-related bloodstream infections. Other manifestations of severe disease are meningitis, endocarditis, osteomyelitis, and surgical and traumatic wound infections. The most common feature in true bacteremia caused by Bacillus is the presence of an intravascular catheter. Herein, we report a case of catheter-related bacteremia caused by B. cereus in a patient with propionic acidemia.