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Children that have a parent with Amyotrophic Lateral Sclerosis (ALS) suffer from the progressive loss of their beloved ones. During the COVID-19 pandemic, the difficulties faced by these children have increased. The study aimed to detect whether there were differences between the minors experiencing a relative's ALS and the minors with no experience of ALS and it aimed also to detect the impact of COVID-19 pandemic on these minors. The study involved Italian participants, in particular: the target group consisted of 38 children (7-18 years) (T0/T1); the control group consisted of 38 children (9-14 years) (T0 only). The following variables were measured: attachment with the Security Scale (SS), affects with the Positive and Negative Affect Schedule for Children (PANAS-C), behavioural problems with Strengths and Difficulties Questionnaire (SDQ), death representation with Testoni Death Representation Scale for Children (TDRS-C), self-concept with the Multidimensional Self Concept Scale (MSCS), resilience and socio-emotional skills with the Devereux Student Strengths Assessment (DESSA). The results showed higher negative affectivity (p < .001), externalising behaviours (p < .05), uncertainty in reflective function (p < .05) in the target group compared to the control one; after the COVID-19 pandemic minors in the target group showed reduced certainty of mental states (p < .05) and interpersonal and scholastic self-esteem (p < .05). The impact of ALS on these minors is significant and produces negative affect, externalizing behaviours and uncertainty of mental states. The lockdown situation due to the COVID-19 pandemic has further aggravated minors in their school and interpersonal self-esteem., (© 2021. The Author(s).)