SRUS is a rare condition in children, which usually presents with a symptom complex of rectal bleeding, passage of mucus and straining on defecation, tenesmus, perineal and abdominal pain, sensation of incomplete defecation, constipation and rectal prolapse. The underlying etiology of SRUS is not fully understood but it is likely to be secondary to ischemic changes in the rectum associated with paradoxical contraction of pelvic floor and external anal sphincter muscles and rectal prolapse. Conservative measures like high intake of fluids and fibers, laxatives, biofeedback and behavior modification therapy may be beneficial for treatment of constipation. Excision of rectal ulcer and surgery of overt rectal prolapse, however, may be required in refractory cases not responding to conservative treatments. A therapeutic role for botulinum toxin injection into the external anal sphincter for treatment of SRUS associated with constipation and paradoxical contraction of pelvic floor and external anal sphincter muscles in children, may exist.