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Summary Objectives The aims of this study were to improve our understanding of the clinical forms of presentation of acute Q fever in Spain and to determine any possible relationships with geographical and seasonal factors. Methods This was a retrospective study of 183 cases of acute Q fever from three Spanish regions, Catalonia, Canary Islands, and La Rioja. Results The main clinical form of presentation was hepatitis (49.2%), followed by isolate febrile syndrome (31.7%) and pneumonia (19.1%). The proportion of cases presenting as pneumonia was significantly higher in La Rioja (40.7%) than in Catalonia (18.3%) or the Canary Islands (12.9%) ( p = 0.001). In Catalonia and the Canary Islands, most cases (52.1% and 57.6%, respectively) were diagnosed between March and June, whereas in La Rioja, most cases (51.8%) occurred between November and February. Overall, the proportion of cases presenting as pneumonia was significantly higher in the period from November to February (32.6%) than in the periods March–June (16.0%) and July–October (13.0%) ( p = 0.01). Conclusions Our results suggest the existence of seasonal differences in the presentation of acute Q fever in Spain, with a higher proportion of pneumonic forms in the colder months. Furthermore, we confirmed the existence of geographical differences, with a higher proportion of pneumonic forms in the region of La Rioja, in the north of the country. [ABSTRACT FROM AUTHOR]