BackgroundMycobacteria avium (M. avium) is a species of ubiquitous slowly growing nontuberculous mycobacteria. It causes opportunistic infections. However, M. avium-related peritonitis in peritoneal dialysis (PD) patients is rare.Case presentationA 51-year-old female end-stage kidney disease patient undergoing PD was admitted for a noninfectious complication. She presented catheter exit site drainage and slightly increased PD effluent white cell count (WCC) with polymorphonuclear predominance on admission. Exit site infection and PD-related peritonitis were diagnosed. Repeated cultures of effluent and drainage were negative. Initial empirical antibiotics and further adjustment were not rewarding. PD was terminated 2 weeks later, however, shortly the patient developed stupor, high fever, peritoneal irritation, and spontaneous chylous ascites, and showed elevated ascitic adenosine deaminase (ADA). The manifestations persisted and the patient’s general condition deteriorated despite intensified antibiotic therapy. Massive parallel sequencing identified M. avium in ascites on hospital day 25, and 4-drug treatment with azithromycin, amikacin, rifampin, and ethambutol was initiated. Nevertheless, the patient died from sepsis on hospital day 30.ConclusionsWe report a case of PD-related M. avium peritonitis. Prolonged culture-negative peritonitis, chylous ascites, and elevated ascitic ADA may hint the possibility of mycobacterial infections. Diagnostic method allowing prompt identification of the pathogen is warranted. The prognosis can be extremely poor, and the prophylaxis and treatment should be better defined.