Hereditary angio-oedema is an autosomal dominant condition resulting in a deficiency of C1 esterase inhibitor protein in serum. The condition is characterized by oedematous attacks in the skin, mucous membranes and gastrointestinal tract triggered by mental and physical stress. We present the rare case of a young girl with known hereditary angio-oedema and spina bifida occulta being admitted for an elective pilonidal sinus excision. This case presented different challenges for the anaesthetists and surgeons.