RESUMEN
Epidermal nevus syndrome is defined as the association of epidermal nevi with abnormalities of the other organ systems, and is regarded as one of the neurocutaneous syndromes. However, till now, epiderrnal nevus syndromi has not been reported with either Morning glory syndrome or intestinal pseudoobstruction. We experienced a case of a 7-year old rnale who had had multiple epidermal nevi on the scalp and forehead and solitary larg cafeau lait spot on his chest since birth. He had histories of failure to thrive and mental retardation, but no seizures. He also had histories of frequent diarrhea and abdominal distension. Physical examination showed abnormalities of the skin, teeth, and Morning glory syndrome of both oplic discs. Colon study, small bowel series and biopsy showed intestinal pseudoobstruction. Skeletal survey revealed a difference between the left upper extremity and the right one.