Hernia around the umbilicus is common in this part of the world. Most are supraumbilical hernias and are usually symptomlcss. In a previous study it was found that most parents would not want surgical repair. Cosmetic desire is most frequcnt indication for surgery in the uncomplicated hernias. Surgery aims at correcting the defect and cstablishing a firm anterior abdominal wall, as is done for large ventral hernias in cases of gastroschisis and exomphalos. Hurler syndrome (Type I mucopolysaccharodosis) is inherited as an autosomal recessive condition. It has a poor prognosis as most of the cases die by the age of 10 years, frequently from myocardial failure? The case presented hcre of a severely mentally retarded child with grotesque and coarse fades is of interest, particularly in the African setting, that in spite of these severe handicaps and apparent poor prognosis, the parents opted for corrective surgery to improve the physical appearance of the child. In a giant hernia, after reduction of the hernia and closure of the sac a definitive repair of the defect has to be achieved. Increased intra-abdominal pressure which is likely to reduce lung compliance should be anticipated and the postoperative period should be closely observed for ventilatory insufficiency.