Naevus syringocystadenomatosus papilliferus with skull and brain lesions, hemiparesis, epilepsy and mental retardation.

Summary— A case of naevus syringocystadenomatosus papilliferus of the left side of the scalp is reported in a severely mentally retarded female, aged 45, who has also an ipsilateral, extensive hyperostosis of the skull, atrophy of the cerebral hemisphere, coloboma of upper eyelid, with an adjacent dermoid growth. There is an area of cortical calcification in the post parietal region. She also suffers from rosacea, right sided hemiparesis, epilepsy and uvula bifida.