Malakoplakia is a rare chronic granulomatous disease, which is highly associated with urinary tract infection, particularly from Escherichia coli. The clinical presentation varies among patients, depending on organ involvement. The course of the disease is usually prolonged and devastating. Because of various clinical manifestations, it is difficult to make the correct diagnosis. There are few patients, thus there is no current properly studied treatment regimen. However, intracellular antibiotics such as ciprofloxacin, or cotrimoxazole, in combination with surgery, are recommended. The clinical presentations of three patients with malakoplakia in Songklanagarind Hospital are reported. Patient 1 presented with a history of urinary tract infection, resulting in unilateral nephrectomy. After surgery, she developed rectal pain and mucous bloody diarrhea. Patient 2 came in with a history of recurrent urinary tract infection and subsequently developed enterocutaneous fistula. Patient 3 came in with urinary tract infection and left ureteric obstruction. In all patients, malakoplakia was diagnosed by tissue biopsy, and all responded well to prolonged antimicrobial therapy. Because patients with malakoplakia suffer significant morbidity and mortality, early detection and appropriate treatment are crucial in improving the outcome.