Desmosis coli is a rare pathology presenting as slow transit constipation. In this case we would like to discuss the presentation and management of desmosis coli. A 14-month-old female hailing from western India with a history of chronic constipation presented with acute massive abdominal distension and vomiting. At laparotomy, a hugely dilated transverse and sigmoid colon with a transition zone at the lower sigmoid was found. A transverse stoma was done after taking multiple sero-muscular biopsies. The patient underwent re-exploration on day 14 because of the non-functioning of the stoma and a fixed bowel loop. The histopathology report was suggestive of normal ganglion cells. Unfortunately, the stoma continued not to function. A dye study showed dye in the colon after 24 hours ruling out any anatomical obstruction. Histopathology slides were reviewed multiple times and reported lack of connective tissue of the colonic wall leading to the diagnosis of desmosis coli. The patient was started on gradual feeds and pro-kinetics and over the next 2 weeks the stoma started functioning slowly. Desmosis coli is a rare cause of constipation which should be suspected in cases where aganglionosis has been ruled out and the constipation is refractory to conventional therapy.