Six children, 4 girls and two boys, aged 5-14 years, with carpal tunnel syndrome (CTS) are reported. Median nerve entrapment had different aetiologies in each case. One patient developed unilateral CTS symptoms after intensive basketball training. He improved upon terminating this sporting activity. In 3 patients bilateral CTS was associated with Schwartz-Jampel syndrome, trigger finger and mucopolysaccharidosis I (MPS IS = Scheie syndrome), respectively. The latter subject, a boy aged 11 years who had severe bilateral muscle thenar weakness and atrophy, made a good recovery after surgery. Two cases with bilateral CTS had autosomal dominant disease. One of them showed familial CTS with thickening of the transverse carpal ligament. The other child (5 years old) presented early bilateral CTS as first manifestation of hereditary neuropathy with liability to pressure palsies (HNPP). His relatives were asymptomatic, but they showed electrophysiological and nerve biopsy changes consistent with HNPP. Nerve conduction studies (NCS) are diagnostic in paediatric CTS. Moreover, NCS is an objective method to evaluate the evolution of the nerve lesions after surgery. NCS must be performed in nerves of the propositus other than the median, as well as in first degree symptomatic and asymptomatic relatives in order to identify possible familial neuropathies.