Hirschsprung's disease is an inherited disorder characterized by the absence of 
ganglion cells in the distal bowel. Neurocutaneous melanosis is a rare 
congenital syndrome characterized by proliferation of melanin-producing cells in 
the skin and leptomeninges. The authors described a newborn patient with 
neurocutaneous melanosis associated with Hirschsprung's disease. This male baby 
had congenital hydrocephalus, large and multiple pigmented skin nevi, and severe 
abdominal distension. He showed marked hydrocephalus at birth and underwent a 
ventriculo-peritoneal shunt at the age of 5 days. Investigations for gut 
motility disorders revealed typical findings consistent with Hirschsprung's 
disease involving the rectosigmoid colon. He was surgically treated for 
Hirschsprung's disease after transanal endorectal pull-through at the age of 7 
months. After settlement of the ventriculo-peritoneal shunt, the transanal 
approach was of significant value for keeping the intraperitoneal catheter 
clean. The association of developmental disorders of melanocytes and enteric 
ganglia, both of which originated from the neural crest, suggested the presence 
of mutual pathogenetic factors in the patient.