Malakoplakia is a rare granulomatous disease of infectious etiology. The name is derived from the Greek malakos (soft) and plakos (plaque), describing its usual clinical presentation as friable yellow soft plaques.

It was first described by Von Hansemann in 1901 and in 1902 by Michaelis and Gutmann. Total number of patients with malakoplakia is fewer than 500. Most patients have genitourinary tract disease.

We report a case of renal malakoplakia with end stage renal disease in a 42 years old female who presented with pain in the right lower abdomen for about 1 month. Investigations revealed a right kidney in the pelvic region which was non functional on DTPA scan. Right sided open nephrectomy was done. Histopathology suggested malakoplakia with end stage renal disease.

This rare entity is associated with a non- specific clinical presentation. In the imaging studies the appearance of the affected kidneys ranges from a normal kidney to an enlarged, nonfunctioning kidney.

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Dr Ashutosh Kumar