Brown bowel syndrome (BBS) is a very rare condition occurring in association with malabsorption syndromes. It is characterised by deposition of granular, brown pigment (lipofuscin or ceroid) in intestinal smooth muscles. Rarely BBS can be complicated by distention of any segment of the bowel. We present a case of BBS associated with massive dilation of first loop of the small intestine and moderate dilation of the sigmoid colon with functional intestinal disturbances requiring surgical intervention in an 11-year-old boy.