Summary The medical records of 11 horses with idiopathic muscular hypertrophy (MH) of the small intestine were reviewed to determine the clinical and pathological features of the disease. The median age of affected horses was 10.0 years (range 5–18 years). No breed or sex predisposition was apparent. Ten horses (91%) had chronic (23 days to 2.4 years) signs of mild, intermittent colic, and 1 horse had signs of severe colic of only 3 days' duration. Partial anorexia and chronic weight loss of variable duration (1–6 months) were prominent historical findings in 5 (45%) horses. Diagnostic tests, with the exception of exploratory caeliotomy, were ineffective for definitive diagnosis of intestinal MH as a cause of colic. In 2 horses, however, a thickened, rigid ileum was detected by palpation per rectum, and in 5 horses, multiple loops of distended small intestine were detected by palpation per rectum. Hypertrophy of both the circular and longitudinal layers of muscularis was determined as the cause of intestinal thickening in all horses. Muscular hypertrophy of the ileum was present in 9 (82%) horses. Two horses (18%) had MH of a section of jejunum only, and 4 (36%) horses had MH of the ileum in combination with MH of other sections of small intestine. Two (18%) horses had MH of the entire small intestine. In 9 (82%) horses, intestinal MH resulted in narrowing of the luminal diameter at the site of MH. Small diverticula were present on the mesenteric border of the hypertrophied ileum of 5 (45%) horses. Five linear (up to 150‐cm) diverticula were present in the hypertrophied jejunum of 1 (9%) horse. Haemomelasma ilei was present on the antimesenteric serosal surface of affected intestine of 8 (73%) horses. Full‐thickness rupture of the ileum with subsequent diffuse, septic peritonitis occurred in 3 (27%) horses.