The number of peritoneal dialysis (PD) catheter-related infections and peritonitis caused by nontuberculous mycobacteria (NTM) has been increasing. Nonetheless, the optimal timing for the relocation of the exit site, removal and reinsertion of the PD catheter, prognosis, and duration of antibiotic treatment remain unclear. This literature review aimed to investigate the epidemiology of patient characteristics and evaluate the most effective diagnostic and treatment strategies for PD catheter-related infections and peritonitis caused by NTM. The systematic literature review was conducted on published cases of PD catheter-related infection and peritonitis caused by NTM in PubMed, Embase, and Ichushi databases up to August 2022. A total of 335 cases (64.1%, male; mean age, 53.4 years; mean dialysis duration, 25.4 months) were analyzed. The most common causative agent of infection was Mycobacterium abscessus (40.1%), followed by Mycobacterium fortuitum (24.8%) and Mycobacterium chelonae (16.6%). With respect to diagnosis, 42.9%, 28.1%, and 29.0% of cases were diagnosed as PD catheter-related infection only, peritonitis only, and both, respectively. The initial cultures were positive for NTM only, positive for any other bacteria, and negative for NTM only in 56.5%, 19.8%, and 23.7% of cases, respectively. Ultimately, more than 80% of cases were treated with multiple antibiotics. PD catheter removal was performed in 55.4% of patients with PD catheter-related infections only and 85.5% of those with PD peritonitis. PD continuation or resumption was possible in 62.2% and 16.0% of patients, respectively. In conclusion, our findings indicate that it is advisable to perform acid-fast bacilli stain and culture in order to promptly identify NTM. PD catheter removal may be an essential management strategy during the early stages of NTM infection.