In order to gain new insight into the pathogenesis and nosography of benign partial epilepsy with occipital paroxysms (BEOP) we retrospectively analysed cases with benign focal sharp waves (SHW) of different localisations, in which analogous EEG changes had been found in at least one first degree relative. Fifty-six families were evaluated. Nineteen cases with occipital SHW (group A) were compared with 37 cases without (group B). There was a broad spectrum of symptomatology with large overlaps between the two groups. However, a number of striking differences, yielding a characteristic picture of early childhood epilepsy with occipital foci were identified: (1) Age of onset under 5 years; (2) a larger proportion of febrile convulsions (FC 47 vs. 19%); (3) a trend towards a higher rate of typical early childhood occipital seizures (26 vs. 5%); (4) a higher rate of frontal and generalizing SHW foci (32 vs. 5%; 37 vs. 11%); (5) a higher rate of generalized spikes and waves (SW) (46 vs. 14%); (6) a trend towards a higher rate of photoparoxysmal response (PPR) (57 vs. 32%). The high prevalence of independent genetic traits favours a multifactorial pathogenesis. The predisposition to FC with characteristic early seizure onset and varying patterns of generalized genetic EEG traits plays a crucial role within the complex pathogenetic network. The early-onset benign childhood occipital seizure susceptibility syndrome of Panayiotopoulos (Benign Childhood Partial Seizures and Related Epileptic Syndromes, John Libbey & Company Ltd., London (1999)) cannot be regarded as the sole representative of occipital spikes in early childhood but as an important even though rare form of occipital epilepsy.