Pneumonia is the main site of infection with Mycoplasma pneumoniae in paediatric age. Nevertheless it can also give rise to other manifestations, with or without respiratory involvement. In the present review are described some unusual clinical features of M. pneumoniae in children. Encephalitis and meningoencephalitis is the most frequent neurological manifestation, but cases of meningitis, myelitis, and polyradiculitis, have been reported. Cardiac involvement is potentially severe, including pericarditis and myocarditis. Cold agglutinin haemolytic anaemia is the most frequent haematologic manifestation. Skin, renal, gastro-intestinal, osteoarticular, and other manifestations have also been reported in the literature. The pathogeny of these extrapulmonary infections is not fully elucidated and the treatment remains partly controversial. Extrapulmonary complications can occur as a result of direct invasion and/or autoimmune response.