The etiology, diagnosis and treatment of childhood granulomatous periorificial dermatitis (GPD) are highly controversial. Some authors underline the similarities between GPD and perioral dermatitis and consider both as part of a spectrum while other authors regard GPD as a distinctive condition. Clinically GPD is a papulo-pustular periorificial disease of the face which histopathologically shows a granulomatous perifollicular infiltrate. Because of its granulomatous pattern, GPD also has been related to cutaneous sarcoidosis. The clinical course is benign and self-limited. Topical steroids are regarded as either the main cause or a worsening factor. While topical treatment is occasionally effective, systemic antibiotic therapy of GPD is usually recommended. We report a "typical" case of GPD and review the literature to discuss the difficulties in its diagnosis and treatment.