A 54-year-old non-right-handed man with positive familial sinistrality showed a pure right hemisphere syndrome following a left hemisphere stroke. Severe right side hemineglect, transcortical motor dysprosodia, spatial dysgraphia and visuo-constructive impairments were observed. At no time were the expected left hemisphere abnormalities such as aphasia, alexia, right-left disorientation or finger agnosia noted. A left fronto-temporal subcortical lesion was documented on CT scan. A Tc-99m HM-PAO SPECT study revealed no cerebral blood flow changes in the right hemisphere while in the left hemisphere a fronto-temporo-parietal cerebral blood flow reduction was evident. This case of a complete reversed laterality of cognitive functions argues for a distinction to be made between 'anomalous' cerebral dominance and 'atypical' cerebral dominance.