AIM: to compare the clinical and diagnostic features and quality of life in patients with different types of idiopathic megabowel. PATIENTS AND METHODS: 157 patients with idiopathic megacolon/megarectum, confirmed by barium enema, were divided on 3 groups: 1) distal idiopathic megabowel (megarectum ± distal third of sigmoid colon dilatation); 2) idiopathic megacolon (variable extent of colon dilatation with a normal size rectum); 3) idiopathic megabowel (megarectum ± variable extent of colon dilatation). Hirschsprung’s disease was excluded in all patients based on complex of clinical features, barium enema and anorectal manometry results and (if needed) rectal Swenson’s biopsy. RESULTS: the cohort included 70 (44.6 %) patients with distal idiopathic megabowel, 50 (31.8 %) patients with idiopathic megacolon and 37 (23.6 %) patients with idiopathic megabowel. Wexner constipation scale rate, rate of integral parameters “abdominal discomfort” and “defecation difficulties”, summary assessment of quality of life by IBSQOL questionnaire did not differ between groups (p > 0.05). At the same time patients with distal idiopathic megabowel were statistically significant younger (p < 0.01), had significant higher rate of faecal incontinence due to faecal impaction overflow (p < 0.01), had less often bowel movement. Also these patients had significant higher rate of distal contrast retention (p < 0.01) during gut transit test, but their colonic transit time was slight faster (p = 0.04).In multivariate analysis both megarectum (OR = 25.42; 95 % CI 5.01–128.92) and insufficiency of anal sphincter (OR = 4.71; 95 % CI 1.38–16.14) were independent predictors of faecal incontinence. The surgical treatment was performed most often in idiopathic megacolon group (p < 0.01), mainly due to colon volvulus. The most patients with distal idiopathic megabowel (97.1 %) were successfully maintained with a conservative treatment. CONCLUSION: there was not substantial difference in clinical features and quality of life in patients with different types of idiopathic megabowel, except of significant higher rate of faecal incontinence and less often bowel movement in distal idiopathic megabowel group. Faecal incontinence in these patients is linked disturbance rather intestinal, than anal component of continence. The necessity in surgical treatment was rising most often in cases of idiopathic megacolon. The conservative treatment was quite effective in most patients with distal idiopathic megabowel.