Little attention has been so far paid to familial cases of Henoch-Schönlein syndrome. We performed a search of the Medical Subject Headings terms (Henoch or Schönlein OR anaphylactoid purpura OR IgA nephropathy OR Berger nephropathy) AND (family OR familial). We identified no more than 19 reports including 47 families with a total of 100 affected cases: their ages ranged from 1.3 to 51 years (median, 11 years), with a male-to-female ratio of 1.4. Familial cases developed simultaneously in 45% and nonsimultaneously in 55% of the families. Age, male-to-female ratio, and clinical findings were not statistically different in cases with simultaneous and nonsimultaneous familial occurrence of Henoch-Schönlein syndrome. Henoch-Schönlein syndrome occurs almost always sporadically. Age at presentation, male-to-female ratio, and findings are similar in familial (both simultaneously and nonsimultaneously occurring) and sporadic Henoch-Schönlein cases.