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Summary This report presents three siblings with neonatal jaundice who died before the age of three months. They were shown on autopsy to be suffering from Niemann‐Pick disease together with a giant cell transformation of the liver. The resemblance of these cases to previously reported examples of the above mentioned double condition is stressed, and a suggestion is made that this variant of the Niemann‐Pick disease presents a special genotypic syndrome within the group with the biochemical disorder broadly classified as sphingomyelin lipidosis.

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