The article reports a rare case of a 30-year-old man with Hirschsprung's disease, who developed a giant fecalith in the rectum and sigmoid (weight 3.5 kg, the largest diameter 20 cm). The fecalith impaired urine flow by compressing urinary tract, thereby causing acute urinary retention and right-sided hydronephrosis with acute obstructive pyelonephritis. Removing fecalith resulted in the patient recovery and normal functioning of genitourinary system.

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