Thirty-two patients (61 limbs) with lysosomal storage disorders underwent surgery for 'carpal tunnel syndrome'. Twenty-two limbs underwent a simple decompression whereas 39 limbs underwent additional neurolysis and tenosynovectomy. Data were incomplete for six patients (12 limbs). The mean age at operation for the decompression group (11 patients) was 10.5 years and for the neurolysis group (15 patients), 6.9 years. Overall, using a neurophysiological rating system, 39% of limbs demonstrated sensory improvement and 47% motor improvement with no significant difference between the groups. Only in the neurolysis group was a decline in motor conduction (2/29 limbs) or sensory conduction (four limbs) noted. Using neurophysiological criteria, no added benefit from external neurolysis was identified.